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1. Lamant L, de Reyniès A, Duplantier MM, Rickman DS, Sabourdy F, Giuriato S, Brugières L, Gaulard P, Espinos E, Delsol G: Gene-expression profiling of systemic anaplastic large-cell lymphoma reveals differences based on ALK status and two distinct morphologic ALK+ subtypes. Blood; 2007 Mar 1;109(5):2156-64
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  • [Title] Gene-expression profiling of systemic anaplastic large-cell lymphoma reveals differences based on ALK status and two distinct morphologic ALK+ subtypes.
  • With the use of microarray gene-expression profiling, we analyzed a homogeneous series of 32 patients with systemic anaplastic large-cell lymphoma (ALCL) and 5 ALCL cell lines.
  • Unsupervised analysis classified ALCL in 2 clusters, corresponding essentially to morphologic subgroups (ie, common type vs small cell and "mixed" variants) and clinical variables.
  • Patients with a morphologic variant of ALCL had advanced-stage disease.
  • Supervised analysis showed that ALK+ALCL and ALK- ALCL have different gene-expression profiles, further confirming that they are different entities.
  • Among the most significantly differentially expressed genes between ALK+ and ALK- samples, we found BCL6, PTPN12, CEBPB, and SERPINA1 genes to be overexpressed in ALK+ ALCL.
  • The molecular signature of ALK- ALCL included overexpression of CCR7, CNTFR, IL22, and IL21 genes but did not provide any obvious clues to the molecular mechanism underlying this tumor subtype.
  • Once confirmed on a larger number of patients, the results of the present study could be used for clinical and therapeutic management of patients at the time of diagnosis.
  • [MeSH-major] Cell Shape. Gene Expression Profiling. Gene Expression Regulation, Neoplastic. Lymphoma, Large-Cell, Anaplastic / genetics. Lymphoma, Large-Cell, Anaplastic / pathology. Protein-Tyrosine Kinases / genetics
  • [MeSH-minor] Cell Line, Tumor. Humans. Immunohistochemistry. RNA, Messenger / genetics. Receptor Protein-Tyrosine Kinases. Tissue Array Analysis


2. Park SR, Baek JY, Kim DW, Im SA, Kim TY, Bang YJ, Kim NK, Jeon YK, Kim CW, Heo DS: Primary systemic anaplastic large cell lymphoma in a single Korean institution: clinical characteristics and treatment outcome. J Korean Med Sci; 2006 Aug;21(4):633-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary systemic anaplastic large cell lymphoma in a single Korean institution: clinical characteristics and treatment outcome.
  • Despite advances in the characterization of anaplastic large cell lymphoma (ALCL), little data is available on Asian patients.
  • We report here upon single Korean institution's experience regarding the clinical characteristics and outcomes of ALCL.
  • We performed a retrospective study of 32 adults with ALCL.
  • Ann Arbor stage III-IV, B symptoms, high-intermediate/ high International Prognostic Index (IPI), and extranodal disease at diagnosis were present in 56%, 44%, 41%, and 63%, respectively.
  • The staining results for anaplastic lymphoma kinase were positive in 6 (33%) of 18 cases available.
  • Age, performance status, lactate dehydrogenase, extranodal disease sites number, and IPI were correlated with treatment response and survival.
  • Our data suggest that Korean ALCL patients appear to have a higher male/female ratio, less frequent skin/bone involvement, and lower CR rate compared with those of Western studies.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / drug therapy. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antigens, CD30 / analysis. Female. Humans. Korea. Male. Middle Aged. Neoplasm Recurrence, Local. Neoplasm Staging. Prognosis. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 16891805.001).
  • [ISSN] 1011-8934
  • [Journal-full-title] Journal of Korean medical science
  • [ISO-abbreviation] J. Korean Med. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Antigens, CD30
  • [Other-IDs] NLM/ PMC2729883
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3. Lowe EJ, Sposto R, Perkins SL, Gross TG, Finlay J, Zwick D, Abromowitch M, Children's Cancer Group Study 5941: Intensive chemotherapy for systemic anaplastic large cell lymphoma in children and adolescents: final results of Children's Cancer Group Study 5941. Pediatr Blood Cancer; 2009 Mar;52(3):335-9
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  • [Title] Intensive chemotherapy for systemic anaplastic large cell lymphoma in children and adolescents: final results of Children's Cancer Group Study 5941.
  • BACKGROUND: Anaplastic large cell lymphoma (ALCL) is characterized by advanced disease at presentation (70-80% of pediatric cases) and accounts for 10-15% of all childhood lymphomas.
  • Treatment strategies for pediatric ALCL vary from short pulse B-NHL chemotherapy to prolonged leukemia like therapy.
  • METHODS: CCG-5941 used a compressed aggressive multiagent T-cell lineage chemotherapy regimen consisting of a 3-week induction therapy (vincristine, prednisone, cyclophosphamide, daunomycin, asparaginase) followed by a 3-week consolidation period (vincristine, prednisone, etoposide, 6-thioguanine, cytarabine, asparaginase, methotrexate) followed by six courses of maintenance chemotherapy at 7-week intervals (cyclophosphamide, 6-thioguanine, vincristine, prednisone, doxorubicin, asparaginase, methotrexate etoposide, cytarabine).
  • RESULTS: Eighty-six children (male 56%, female 44%) with non-localized ALCL (CD30+) were treated.
  • The majority of tumors were positive for ALK (90%) and of T lineage (83%).
  • Extranodal disease was common (mediastinum 35%, skin 15%, lung 14%, bone 12%, bone marrow 13%, liver 6%, and other viscera 17%).
  • Relapse occurred early with 17 (81%) relapses occurring within 2 years of diagnosis and 12 (57%) while receiving therapy.

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  • (PMID = 18985718.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U10 CA098543; None / None / / U10 CA098543-06; United States / NCI NIH HHS / CA / U10 CA098543-06
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS123401; NLM/ PMC2769495
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4. Kaliyadan F, Ray S, Mathew MK, Pai S, Sasikala L, Pai R: A rapidly progressing, fatal case of primary systemic anaplastic large cell lymphoma presenting as erythroderma--association with carbamazepine. Dermatol Online J; 2008;14(12):5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A rapidly progressing, fatal case of primary systemic anaplastic large cell lymphoma presenting as erythroderma--association with carbamazepine.
  • A 73-year-old male patient admitted with erythroderma was diagnosed to have primary systemic Analpastic Lymphoma Kinase (ALK) positive, CD 30 positive, anaplastic large cell lymphoma.
  • The patient's condition deteriorated rapidly during the period after the diagnosis was confirmed, with subsequent death before chemotherapy could be started.
  • Here we highlight the possibility of carbamazepine inducing anaplastic large cell lymphomas and the need for a high level of suspicion to make an early diagnosis allowing rapid appropriate treatment in such cases.
  • [MeSH-major] Carbamazepine / adverse effects. Dermatitis, Exfoliative / complications. Lymphoma, Large-Cell, Anaplastic / diagnosis. Lymphoma, Large-Cell, Anaplastic / etiology
  • [MeSH-minor] Aged. Diabetic Neuropathies / drug therapy. Disease Progression. Fatal Outcome. Humans. Male. Tomography, X-Ray Computed

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  • (PMID = 19265618.001).
  • [ISSN] 1087-2108
  • [Journal-full-title] Dermatology online journal
  • [ISO-abbreviation] Dermatol. Online J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 33CM23913M / Carbamazepine
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5. Belousova IE, Kazakov DV, Sosna B, Sulc M, Michal M: [Small-cell variant of CD30+ -anaplastic large-cell lymphoma of the skin]. Arkh Patol; 2008 Mar-Apr;70(2):40-3
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  • [Title] [Small-cell variant of CD30+ -anaplastic large-cell lymphoma of the skin].
  • Three cases of the so-called variant of primary cutaneous CD30+ anaplastic large cell lymphoma (ALCL) are presented.
  • Microscopically, the tumors were composed of small cells with irregular nuclei that were immunohistochemically positive for CD3, CD5, CD7, and CD30 and negative for B-cell markers; there was focal ALK-1 positivity in 1 case.
  • Fhedium to large CD30+ cells were rarely found scattered in the infiltrate.
  • Chemotherapy was performed and finally the patient underwent allogenic bone marrow transplantation; he died 3 years after the original diagnosis due to acute graft-versus-host disease and sepsis.
  • [MeSH-major] Antigens, CD30. Lymphoma, Large B-Cell, Diffuse / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Aged. Antigens, CD / immunology. Cell Size. Gene Rearrangement, B-Lymphocyte / immunology. Gene Rearrangement, T-Lymphocyte / immunology. Humans. Immunoglobulin Heavy Chains / immunology. Male. Middle Aged. Receptors, Antigen, T-Cell / immunology


6. Kitamura N, Katagiri YU, Itagaki M, Miyagawa Y, Onda K, Okita H, Mori A, Fujimoto J, Kiyokawa N: The expression of granulysin in systemic anaplastic large cell lymphoma in childhood. Leuk Res; 2009 Jul;33(7):908-12
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  • [Title] The expression of granulysin in systemic anaplastic large cell lymphoma in childhood.
  • The expression of granulysin, a cytolytic protein produced by activated T and NK cells, has been revealed to be correlated with the prognosis of some adult cancer patients.
  • By examination on various childhood lymphoma tissues, we found that granulysin level was especially high in systemic anaplastic large cell lymphoma (ALCL) cases, whereas no close correlation with the expression of CD96, a marker for activated T and NK cells, was observed.
  • We further demonstrated that both ALCL cells in biopsy specimens and cell lines established from ALCL express granulysin, indicating some correlation of granulysin with biological features of ALCL.
  • [MeSH-major] Antigens, Differentiation, T-Lymphocyte / genetics. Burkitt Lymphoma / genetics. Hodgkin Disease / genetics. Lymphoma, B-Cell / genetics. Lymphoma, Large B-Cell, Diffuse / genetics. Lymphoma, Large-Cell, Anaplastic / genetics. RNA, Messenger / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Antigens, CD / genetics. Antigens, CD / metabolism. Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Female. Flow Cytometry. Humans. Killer Cells, Natural / metabolism. Killer Cells, Natural / pathology. Male. Middle Aged. Prognosis. Reverse Transcriptase Polymerase Chain Reaction. Tumor Cells, Cultured. Young Adult

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  • (PMID = 19243819.001).
  • [ISSN] 1873-5835
  • [Journal-full-title] Leukemia research
  • [ISO-abbreviation] Leuk. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, Differentiation, T-Lymphocyte; 0 / Biomarkers, Tumor; 0 / CD96 antigen; 0 / GNLY protein, human; 0 / RNA, Messenger
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7. Wang FH, Li YH, Zeng J, Rao HL, Xia ZJ, Sun XF, Huang HQ, Lin TY, Jiang WQ, Guan ZZ: Clinical analysis of primary systemic anaplastic large cell lymphoma: a report of 57 cases. Ai Zheng; 2009 Jan;28(1):49-53
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical analysis of primary systemic anaplastic large cell lymphoma: a report of 57 cases.
  • BACKGROUND AND OBJECTIVE: The clinical characteristics and prognosis of primary systemic anaplastic large cell lymphoma (ALCL) are various according to different reports.
  • Anaplastic lymphoma kinase (ALK), a specific marker of primary systemic ALCL, is related with its clinical characteristics and prognosis.
  • This study was to investigate the clinical characteristics and prognosis of primary systemic ALCL, and to explore the expression and clinical significance of ALK.
  • METHODS: Clinical data of 57 primary systemic ALCL patients, treated in Cancer Center of Sun Yat-sen University from January 1997 to January 2006, were reviewed.
  • Of the 57 patients, 33 (57.9%) had B symptoms, 23 (40.4%) had Ann Arbor stage III-IV tumors, and 23 (40.4%) had extranodal disease at diagnosis.
  • The positive rate of ALK was 63.0% in the 46 cases detected.
  • Compared with ALK-negative patients, ALK-positive patients were younger (p = 0.001) and had higher expected 5-year survival rate (p < 0.01).
  • Multivariate analysis confirmed the independent prognostic values of ALK expression, primary disease site and lactate dehydrogenase (LDH) level.
  • CONCLUSIONS: Primary systemic ALCL usually occurs in young patients, with good response to chemotherapy and good prognosis.
  • No ALK expression, high-intermediate/high IPI, extranodal disease and elevated LDH level are correlated to unfavorable prognosis.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / drug therapy. Protein-Tyrosine Kinases / analysis

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  • (PMID = 19448428.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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8. Gualco G, Weiss LM, Bacchi CE: Expression of p63 in anaplastic large cell lymphoma but not in classical Hodgkin's lymphoma. Hum Pathol; 2008 Oct;39(10):1505-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Expression of p63 in anaplastic large cell lymphoma but not in classical Hodgkin's lymphoma.
  • Immunohistochemical determination of p63 protein is frequently used in the pathologic diagnosis of nonhematological solid tumors.
  • In malignant hematological disease, p63 expression has been reported in 22% of follicular lymphoma, about 35% of diffuse large B-cell lymphoma, 23% of chronic lymphocytic leukemia, and in some cases of blast crisis of chronic myelogenous leukemia.
  • Anaplastic large cell lymphoma is a rare disease that accounts for less than 5% of all cases of non-Hodgkin's lymphoma.
  • There is little information concerning p63 expression in this specific type of lymphoma.
  • In some cases, the morphological and phenotypic features between anaplastic large cell lymphoma and classical Hodgkin's lymphoma are similar, making this differential diagnosis challenging.
  • We studied p63 expression using a tissue microarray approach in 154 cases of anaplastic large cell lymphoma, including 38% anaplastic large cell kinase positive and 62% anaplastic large cell kinase negative, and 58 Hodgkin's lymphoma cases.
  • Sixty-eight cases of anaplastic large cell lymphoma (44%) showed p63 nuclear positivity (41% of anaplastic large cell kinase positive and 47% of anaplastic large cell kinase negative).
  • Of 130 cases of systemic-anaplastic large cell lymphoma, 42% showed p63 positivity.
  • The neoplastic cells expressed p63 in 38% of the cases of CD45-negative/anaplastic large cell kinase-negative null cell-type anaplastic large cell lymphoma, a subgroup that offers the most difficulties in the differential diagnosis with classical Hodgkin's lymphoma.
  • In contrast, none of the cases of classical Hodgkin's lymphoma demonstrated any p63 expression.
  • These results demonstrate that p63 protein expression is frequently expressed in a subset of anaplastic large cell lymphoma cases and may be used as a potential tool in the differential diagnosis between anaplastic large cell lymphoma and classical Hodgkin's lymphoma.

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  • (PMID = 18620733.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA112217-03; United States / NCI NIH HHS / CA / R01 CA112217; United States / NCI NIH HHS / CA / 5R01CA082274; United States / NCI NIH HHS / CA / R01 CA112217-03; United States / NCI NIH HHS / CA / R01 CA082274-08; United States / NCI NIH HHS / CA / CA082274-08; United States / NCI NIH HHS / CA / R01 CA082274; United States / NCI NIH HHS / CA / U01 CA121947-016821; United States / NCI NIH HHS / CA / 5R01CA112217; United States / NCI NIH HHS / CA / CA121947-016821
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / CKAP4 protein, human; 0 / Membrane Proteins; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
  • [Other-IDs] NLM/ NIHMS127050; NLM/ PMC2744879
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9. Shi Y, Chen G, Zhou XG, Gong LP, Yu R, Zheng YY, Xie JL, Jin Y: [Clinicopathologic features of 66 cases of anaplastic lymphoma kinase positive and negative systemic anaplastic large cell lymphoma: a comparative study]. Zhonghua Bing Li Xue Za Zhi; 2010 Apr;39(4):235-9
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  • [Title] [Clinicopathologic features of 66 cases of anaplastic lymphoma kinase positive and negative systemic anaplastic large cell lymphoma: a comparative study].
  • OBJECTIVE: To study the clinicopathologic features of 66 cases of primary systemic anaplastic large cell lymphoma (ALCL), with emphasis on the differences between ALK-positive and ALK-negative cases.
  • METHODS: The clinical data of 66 cases of ALCL was analyzed.
  • Immunohistochemical study for CD30, ALK protein, epithelial membrane antigen, CD2, CD3, granzyme B and TIA-1 was carried out.
  • The differences between ALK-positive and ALK-negative cases were statistically analyzed.
  • RESULTS: There were 48 cases of ALK-positive ALCL and 18 cases of ALK-negative ALCL.
  • The patients with ALK-positive ALCL were younger than those with ALK-negative ALCL (P < 0.05), with the median age being 18 years and 36 years, respectively.
  • Fever, especially hyperpyrexia, was more commonly observed in ALK-positive ALCL patients than in ALK-negative ALCL patients (33 cases versus 4 cases, P < 0.05).
  • The overall survival rate and median duration of survival in patients with ALK-positive ALCL were higher and longer than those in patients with ALK-negative ALCL (80% versus 71%; 21 months versus 12.5 months, P > 0.05).
  • There were however no significant differences in histology between ALK-positive ALCL and ALK-negative ALCL.
  • "Hallmark" cells were seen in most of the ALCL cases.
  • Most ALK-positive cases belonged to the common variant (35 cases).
  • Small cell variant and sarcomatoid subtype were found only in few cases (3 cases and 2 cases, respectively).On the other hand, common variant (17 cases) constituted the majority of ALK-negative ALCL.
  • Immunohistochemical study showed that ALK-positive ALCL always expressed CD30 and epithelial membrane antigen.
  • ALK-positive ALCL more often expressed epithelial membrane antigen (100% versus 72%; P < 0.05) but less so for T-cell markers (including CD2, CD3, CD43 and CD45RO).
  • Cytotoxic molecules were more commonly expressed in ALK-positive ALCL (P > 0.05).
  • FISH showed that in ALK-positive ALCL, 1 case had normal ALK gene, 1 had deletion and multicopy and 2 had deletion.
  • On the other hand, 1 case of ALK-negative ALCL had normal ALK gene.
  • CONCLUSIONS: While there are no significant morphologic differences between ALK-positive ALCL and ALK-negative ALCL, the clinical features, immunophenotypes and genetic features of both groups vary.
  • These differences are helpful in guiding the differential diagnosis.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / metabolism. Lymphoma, Large-Cell, Anaplastic / pathology. Protein-Tyrosine Kinases / metabolism
  • [MeSH-minor] Adolescent. Adult. Age Factors. Antigens, CD30 / metabolism. Child. Child, Preschool. Diagnosis, Differential. Female. Follow-Up Studies. Gene Deletion. Humans. Male. Malignant Hyperthermia / etiology. Middle Aged. Mucin-1 / metabolism. Neoplasm Recurrence, Local. Receptor Protein-Tyrosine Kinases. Survival Rate. Young Adult

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  • (PMID = 20654121.001).
  • [ISSN] 0529-5807
  • [Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology
  • [ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi
  • [Language] chi
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Antigens, CD30; 0 / Mucin-1; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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10. Goteri G, Simonetti O, Rupoli S, Piccinini G, Rubini C, Stramazzotti D, Fazioli F, Capomagi C, Leoni P, Offidani AM, Lo Muzio L: Differences in survivin location and Bcl-2 expression in CD30+ lymphoproliferative disorders of the skin compared with systemic anaplastic large cell lymphomas: an immunohistochemical study. Br J Dermatol; 2007 Jul;157(1):41-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Differences in survivin location and Bcl-2 expression in CD30+ lymphoproliferative disorders of the skin compared with systemic anaplastic large cell lymphomas: an immunohistochemical study.
  • BACKGROUND: Cutaneous CD30+ lymphoproliferative disorders (LPDs) are a spectrum of disease associated with a favourable prognosis.
  • Systemic anaplastic large cell lymphoma (ALCL), although morphologically and phenotypically similar, differs in clinical presentation and has a less favourable biological behaviour.
  • Dysregulation of apoptosis, the process regulating cell population by programmed death, can explain the differences among these disorders.
  • OBJECTIVES: We investigated the expression of two inhibitors of apoptosis, survivin and Bcl-2 protein, in serial skin lesion samples from CD30+ LPDs compared with systemic ALCL.
  • METHODS: Immunohistochemical analysis with antibodies against anaplastic lymphoma kinase (ALK)-1 protein, survivin and Bcl-2 protein was performed in 10 cutaneous CD30+ LPDs (five lymphomatoid papulosis, five ALCL) and 18 systemic ALCLs.
  • RESULTS: Cutaneous CD30+ LPDs shared a heterogeneous expression of cytoplasmic survivin with all systemic ALCLs, and of Bcl-2 with systemic ALK- ALCLs; however, they differ from systemic ALK- ALCLs because they lack nuclear survivin (P = 0.045), and from systemic ALK+ ALCLs by a higher expression of Bcl-2 (P = 0.045) and a lack of ALK-1.
  • Overall, coexpression of Bcl-2 and nuclear survivin in CD30+ LPDs was associated with a less favourable disease survival.
  • CONCLUSIONS: The different patterns of expression of Bcl-2 and survivin in CD30+ LPDs might have an impact on their different biological and clinical behaviour.
  • Moreover, nuclear localization of survivin, similarly to ALK, may be a useful marker for predicting a systemic form of ALCL with cutaneous presentation.
  • [MeSH-major] Antigens, CD30 / metabolism. Lymphoproliferative Disorders / diagnosis. Microtubule-Associated Proteins / metabolism. Neoplasm Proteins / metabolism. Proto-Oncogene Proteins c-bcl-2 / metabolism. Skin Neoplasms / diagnosis
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Cysteine Proteinase Inhibitors / pharmacology. Diagnosis, Differential. Female. Humans. Immunohistochemistry / methods. Inhibitor of Apoptosis Proteins. Lymphoma, Large B-Cell, Diffuse / diagnosis. Male. Middle Aged. Protein-Tyrosine Kinases / metabolism. Receptor Protein-Tyrosine Kinases. Skin

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  • [ErratumIn] Br J Dermatol. 2007 Aug;157(2):431. Lomuzio, L [corrected to Lo Muzio, L]
  • (PMID = 17484779.001).
  • [ISSN] 0007-0963
  • [Journal-full-title] The British journal of dermatology
  • [ISO-abbreviation] Br. J. Dermatol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD30; 0 / BIRC5 protein, human; 0 / Cysteine Proteinase Inhibitors; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Neoplasm Proteins; 0 / Proto-Oncogene Proteins c-bcl-2; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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11. Huang W, Li X, Yao X, Lu Y, Li B, Sheng W, Lu H, Jin A, Zhou X: Expression of ALK protein, mRNA and fusion transcripts in anaplastic large cell lymphoma. Exp Mol Pathol; 2009 Apr;86(2):121-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Expression of ALK protein, mRNA and fusion transcripts in anaplastic large cell lymphoma.
  • Systemic anaplastic large cell lymphoma (ALCL) can be divided into two subgroups, anaplastic lymphoma kinase (ALK)-positive and ALK-negative, based on the expression of ALK protein.
  • In this study, we investigated the expression of ALK protein, mRNA and fusion transcripts involving ALK and their relationships in ALCL and analyzed formalin-fixed, paraffin-embedded tissues.
  • Our results showed that the expression of ALK protein, ALK mRNA and ALK fusion transcripts were significantly related to one another (P<0.01).
  • Consistent with the expression of ALK protein, patients presenting with ALK mRNA or ALK involved fusion transcripts were significantly younger than those lacking ALK gene alteration (P<0.01).
  • Combined detection of ALK protein, ALK mRNA and ALK fusion transcripts can complement each other to aid in the diagnosis of ALCL.
  • [MeSH-major] Gene Expression Regulation, Neoplastic. Lymphoma, Large-Cell, Anaplastic / enzymology. Lymphoma, Large-Cell, Anaplastic / genetics. Oncogene Proteins, Fusion / genetics. Protein-Tyrosine Kinases / genetics

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  • (PMID = 19135051.001).
  • [ISSN] 1096-0945
  • [Journal-full-title] Experimental and molecular pathology
  • [ISO-abbreviation] Exp. Mol. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / RNA, Messenger; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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12. Summers TA, Moncur JT: The small cell variant of anaplastic large cell lymphoma. Arch Pathol Lab Med; 2010 Nov;134(11):1706-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The small cell variant of anaplastic large cell lymphoma.
  • Anaplastic large cell lymphomas constitute a heterogeneous group of hematopoietic neoplasms that are characterized by immunopositivity for CD30 and the presence, in varying degrees, of large, pleomorphic "hallmark" cells.
  • Primary systemic anaplastic lymphoma kinase-positive anaplastic large cell lymphomas are a subset of this group.
  • Numerous heterogeneous histomorphologic patterns have been described in anaplastic lymphoma kinase-positive anaplastic large cell lymphomas, and all patterns tend to have a better prognosis than that found in anaplastic lymphoma kinase-negative cases.
  • We provide a short review of the small cell variant of anaplastic large cell lymphoma to facilitate the diagnosis of this difficult-to-recognize entity, which may be confused with reactive processes, commonly presents with disseminated disease, and pursues an aggressive clinical course.
  • [MeSH-major] Lymphocytes / pathology. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Antigens, CD30 / metabolism. Diagnosis, Differential. Humans

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  • (PMID = 21043827.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30
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13. Andorsky DJ, Yamada R, Steward K, De Vos S, Said J, Timmerman J: Expression of programmed death ligand 1 (PD-L1) by non-Hodgkin's lymphomas (NHL) and effect on tumor-associated T cells. J Clin Oncol; 2009 May 20;27(15_suppl):8526

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Expression of programmed death ligand 1 (PD-L1) by non-Hodgkin's lymphomas (NHL) and effect on tumor-associated T cells.
  • METHODS: PD-L1 expression was analyzed in 16 NHL cell lines by flow cytometry (FC) and in 111 lymphoma specimens by immunohistochemistry (IHC) (n=92) or FC (n=19).
  • In functional studies, irradiated anaplastic large cell lymphoma (ALCL) cells were co-cultured with allogeneic T cells in the presence of anti-PD-L1 blocking antibody, and IFNγ secretion and thymidine incorporation was used to assess T cell function and proliferation.
  • To further test tumor-T cell interactions, malignant ascites from a patient with ALK+ ALCL and peripheral blood mononuclear cells from a patient with leukemic mantle cell lymphoma, both containing PD-L1-expressing tumor cells and tumor-associated T cells, were stimulated with phytohemagglutinin (a polyclonal T cell activator) and incubated with anti-PD-L1 antibody.
  • Levels of 16 inflammatory cytokines were measured as an assessment of T cell activity.
  • RESULTS: All 9 B cell lymphoma lines were negative for PD-L1, while all 5 ALCL cell lines were strongly positive.
  • One T-cell ALL line was positive, and one peripheral T cell lymphoma was negative.
  • Strong PD-L1 staining was detected by IHC in all 14 ALCL specimens and in 83% of diffuse large B cell lymphomas (DLBCL) analyzed (n=35).
  • Activity of allogeneic T cells co-cultured with irradiated ALCL cells, as measured by IFNγ secretion and proliferation, was markedly enhanced in the presence of anti-PD-L1 blocking antibody.
  • In the autologous setting using cultures of ALCL and mantle cell lymphoma specimens containing host T cells, secretion of inflammatory cytokines by tumor-associated T cells, including GMCSF, IFNγ, IL-1, IL-6, IL-8, TNFα, and MIP1α, were increased by incubation with anti-PD-L1 antibody.
  • CONCLUSIONS: PD-L1 is highly expressed in ALCL and in a majority of DLBCL.
  • PD-L1 may play a role in thwarting an effective anti-tumor immune response and represents an attractive target for lymphoma immunotherapy.

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  • (PMID = 27960901.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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14. Castillo J, Milani C, Pantanowitz L: HIV-associated anaplastic large cell lymphoma. J Clin Oncol; 2009 May 20;27(15_suppl):e19563

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] HIV-associated anaplastic large cell lymphoma.
  • : e19563 Background: Anaplastic large cell lymphoma (ALCL) is a CD30+ T-cell lymphoma that is generally unrelated to EBV in the non-HIV setting.
  • Based upon anaplastic lymphoma kinase (ALK) expression, the new WHO classification provisionally distinguishes between ALK+ (favorable) and ALK- (unfavorable) ALCL.
  • The characteristics of ALCL, such as ALK expression and EBV coinfection, in individuals with HIV infection have not been adequately evaluated.
  • The aim of this study was to investigate these features in HIV-associated ALCL cases.
  • METHODS: A MEDLINE search for all cases of HIV-associated non-cutaneous ALCL was undertaken.
  • Data regarding patient age, gender, HIV status (CD4 count, viral load, opportunistic infections), HAART, lymphoma features (B symptoms, stage, sites of involvement, immunophenotype, ALK expression, molecular studies), EBV coinfection, therapy and outcome (survival, cause of death) were extracted and analyzed.
  • ALCL was extranodal in 22 cases (96%) affecting most commonly lung, soft tissue and liver.
  • Many (78%) patients had stage IV disease and B symptoms were reported in 9 cases (50%).
  • T-cell receptor gene rearrangement was present in all cases, CD30 was positive in 22 (96%), and the vast majority (90%) were ALK-negative.
  • Therapy for ALCL was documented in 15 (67%) cases; 64% received CHOP.
  • Death was caused by either lymphoma progression (42%) or infection (58%).
  • CONCLUSIONS: HIV-associated non-cutaneous ALCL appears to affect younger individuals and is associated with EBV infection in a subset of cases.
  • Apart from marked immunosuppression, the poor prognosis of HIV-associated ALCL appears to be related to the absence of ALK expression, advanced stage at presentation with prominent extranodal disease, inadequate therapy including HAART, and poor response to CHOP.
  • Further research is needed to better understand and treat this unique HIV-associated lymphoma.

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  • (PMID = 27961064.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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15. Vera L, Reategui R, Beltran B, Morales D, Capellino A, Desposorio C, Castillo J: The clinicopathological spectrum of HIV-associated lymphoma: Eleven-year-experience in a general hospital in Peru. J Clin Oncol; 2009 May 20;27(15_suppl):e19561

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The clinicopathological spectrum of HIV-associated lymphoma: Eleven-year-experience in a general hospital in Peru.
  • : e19561 Background: The clinicopathological spectrum of HIV-associated lymphomas in developing countries has not been clearly defined.
  • METHODS: This is a retrospective review of the clinical records of patients with diagnosis of HIV in our institution from March 1997 to March 2008.
  • RESULTS: Forty-eight patients with HIV-associated lymphoma were identified.
  • Forty-four cases (92%) were diagnosed with non-Hodgkin lymphoma (NHL) and 4 cases (8%) with Hodgkin lymphoma (HL).
  • From the 44 NHL cases, 40 cases (91%) were of B-cell origin; 23 cases (57.5%) had diffuse large B-cell, 9 cases (22.5%) had Burkitt, 3 cases (7.5%) had plasmablastic, 2 cases (5%) had primary CNS, 2 cases (5%) had MALT and 1 case (2.5%) had primary effusion lymphoma.
  • The remaining 4 cases (9%) were of T-cell origin; 3 cases (75%) had peripheral T-cell lymphoma NOS and 1 case (25%) was ALK-negative anaplastic large cell lymphoma.
  • Only 16 patients (33%) were receiving HAART previously the diagnosis of NHL and 33 patients (68%) received any oncology treatment.
  • Also a high proportion of T-cells lymphomas are found.

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  • (PMID = 27961062.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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16. Goldman S, Coiffier B, Reiter A, Younes A, Cairo MS, International TLS Expert Panel: A medical decision tree for the prophylaxis (P) and treatment (T) of tumor lysis syndrome (TLS): An international TLS consensus panel. J Clin Oncol; 2009 May 20;27(15_suppl):e17575

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Additionally, we recently reported an evidence based review of guidelines for the P and T of TLS (Coiffier et al, J Clin Oncol. 2008).
  • 2001) and a rapid reduction in UA in adults at high-risk of TLS (Coiffier et al, J Clin Oncol. 2003).
  • METHODS: We convened an international panel (N = 17) of experts in pediatric and adult hematological malignancies and solid tumors (ST) to develop a medical decision tree for the P and T of TLS based on the risk classification (low, medium, high) and management recommendations of Coiffier et al (J Clin Oncol.
  • 2008) Results: Patients without evidence of LTLS were assigned to either low-risk disease (LRD), medium-risk (MRD), or high-risk (HRD).
  • Risk factors included pathological classification stage, bulk, disease burden (WBC/LDH) and renal impairment/involvement.
  • MRD consisted of ALL ≤100K/mm<sup>3</sup>, AML 25-100K/mm<sup>3</sup>, BL/LL stage I/II and low LDH, childhood ALCL, DLBCL/PTCL/MCL/ATL non-bulky but elevated LDH, CLL treated with targeted therapy, and LRD with renal impairment/involvement.
  • CONCLUSIONS: This medical decision tree will facilitate the practice of management of the P and T of TLS and hopefully improve the quality of care in a cost effective manner.

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  • (PMID = 27963935.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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17. Kwak EL, Camidge DR, Clark J, Shapiro GI, Maki RG, Ratain MJ, Solomon B, Bang Y, Ou S, Salgia R: Clinical activity observed in a phase I dose escalation trial of an oral c-met and ALK inhibitor, PF-02341066. J Clin Oncol; 2009 May 20;27(15_suppl):3509

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Tumor types included colorectal, pancreatic, sarcoma, ALCL and NSCLC.
  • Among 10 NSCLC pts whose tumors harbor EML4-ALK rearrangement, 1 pt has had a PR, 2 pts have achieved unconfirmed PR and 4 pts have had SD (3 have experienced reduction in tumor burden by ∼20% in measurable lesions and 1 has been treated for 28 weeks).

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  • (PMID = 27961297.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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18. Blum KA, Smith M, Fung H, Zalevsky J, Combs D, Ramies DA, Younes A: Phase I study of an anti-CD30 Fc engineered humanized monoclonal antibody in Hodgkin lymphoma (HL) or anaplastic large cell lymphoma (ALCL) patients: Safety, pharmacokinetics (PK), immunogenicity, and efficacy. J Clin Oncol; 2009 May 20;27(15_suppl):8531

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Phase I study of an anti-CD30 Fc engineered humanized monoclonal antibody in Hodgkin lymphoma (HL) or anaplastic large cell lymphoma (ALCL) patients: Safety, pharmacokinetics (PK), immunogenicity, and efficacy.
  • : 8531 Background: XmAb2513 is a novel 2<sup>nd</sup>-generation humanized monoclonal antibody (mAb) directed against CD30 (a cell surface antigen expressed on Reed-Sternberg cells of HL and ALCL), with an Fc region engineered to have increased binding affinity to Fcγ receptors (FcγRs) leading to improved FcγR-dependent effector cell functions.
  • In vitro, XmAb2513 was more potent and more efficacious than 1<sup>st</sup>-generation anti-CD30 mAbs (e.g., SGN-30 & MDX-060).
  • A Phase 1 study has been initiated to examine the safety and efficacy of XmAb2513 in patients (pts) with relapsed HL and ALCL.
  • Pts receive up to 4 cycles (8 infusions) with sample collections at regular intervals for safety, PK, soluble CD30 (sCD30) and detection of immunogenicity [i.e., human Abs to humanized Abs (HAHA)].
  • Half-life appeared to increase after multiple infusions and ranged from 5.3 - 30.2 days in the 1 and 3 mg/kg cohorts.

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  • (PMID = 27960926.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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19. Oki M, Isozaki M, Nakamura N, Kikuchi A, Tsuchiya T, Arbogast P, Ogawa Y, Ando K: A multivariate analysis for the survival of nodal peripheral T-cell lymphoma (PTCL). J Clin Oncol; 2009 May 20;27(15_suppl):e19521

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A multivariate analysis for the survival of nodal peripheral T-cell lymphoma (PTCL).
  • : e19521 Background: Nodal peripheral T-cell lymphoma (PTCL) is uncommon lymphoma with various subtypes and poor prognosis.
  • However, the superiority of high-dose chemotherapy followed by autologous stem cell transplantation (ASCT) has remained inconclusive.
  • The infrequency of PTCL is an obstacle to perform a large prospective study.
  • We conducted a retrospective study of fifty six patients with three major nodal PTCL, histologically classified as PTCL-not specified (NOS, n=29), angioimmunoblastic T-cell lymphoma (AITL, n=19), and ALK-negative anaplastic large cell lymphoma (ALCL, n=8) who underwent ASCT (n=14) or not (n=42) after CT in Tokai University Hospital, Ebina General Hospital, and Hadano Red Cross Hospital, Kanagawa, Japan between 1997 and 2008.
  • Hazard ratio (HR) of ASCT, IPI, and sIL-2R were 0.24 (95%CI: 0.08-0.76, p=0.02), 0.77 (95%CI: 0.34-1.77, p=0.54), and 1.45 (95%CI: 1.129-1.857, p=0.035), respectively.

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  • (PMID = 27960935.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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20. Schmitz N, Ziepert M, Nickelsen M, Wolf SP, Truemper L, Loeffler M, Ho A, Metzner B, Rosenwald A, Pfreundschuh M: Mature T-/NK-cell lymphomas: Prognostic factors and treatment outcome of patients treated on studies of the German High-Grade Lymphoma Study Group (DSHNHL). J Clin Oncol; 2009 May 20;27(15_suppl):8564

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mature T-/NK-cell lymphomas: Prognostic factors and treatment outcome of patients treated on studies of the German High-Grade Lymphoma Study Group (DSHNHL).
  • : 8564 Background: T-cell lymphomas represent a heterogeneous group of malignancies difficult to diagnose and to treat.
  • METHODS: Between 1993 and 2006 we treated 329 pts with ALK-positive ALCL (73 pts), ALK-negative ALCL (108 pts), PTCL, NOS (68 pts), AITL (28 pts), NK-/T-cell lymphoma (18 pts), and rare T-cell lymphomas on prospective studies.
  • RESULTS: The majority of pts with ALK-positive ALCL presented with IPI 0, 1 (62%) or IPI 2 (26%) and had an excellent overall survival (OS) of 89 % and event-free survival (EFS) of 75% at 3 yrs.
  • OS, EFS were significantly better for ALK-positive ALCL but did not significantly differ for pts in other histological subgroups.
  • In younger pts (ALK-positive ALCL were excluded) and good-risk disease (LDH <= N) there was a trend for better EFS after the addition of E to CHOP (EFS 63% vs. 48%, p = 0.065).
  • The MegaCHOEP protocol (Schmitz et al., CANCER 2006) failed to improve treatment results for younger pts with poor-risk disease (EFS at 3 yrs: 25.9%, 95% CI: 10.4-41.4); the prospective study comparing MegaCHOEP with CHOEP-14 was stopped for pts with T-cell lymphoma.
  • CONCLUSIONS: CHO(E)P results in excellent OS of pts with ALK-positive ALCL and selected pts with other histologies and low IPI.

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  • (PMID = 27961019.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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21. Lansigan F, Cooper D, Seropian S, Foss F: Autologous and allogeneic transplantation for aggressive T-cell lymphomas: A single institution experience. J Clin Oncol; 2009 May 20;27(15_suppl):8558

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Autologous and allogeneic transplantation for aggressive T-cell lymphomas: A single institution experience.
  • : 8558 Aggressive T-cell lymphomas (ATCL) represent 10-15% of non-Hodgkin lymphoma and have a worse prognosis than aggressive B-cell lymphomas.
  • Both autologous (Auto) and allogeneic (Allo) stem cell transplantation have been used as consolidation in first remission and at relapse, but the role of transplantation has not been clearly defined.
  • The Allo group consisted of 4 PTCLu, 3 angioimmunoblastic(AITL), 2 panniculitis-like, 2 cutaneous(CTCL) with large cell transformation, 2 NK-cell, 2 anaplastic large cell(ALCL), 1 hepatosplenic, 1 enteropathic, and 1 refractory CTCL.
  • The Auto group consisted of 6 PTCLu, 12 ALCL (5 Alk+, 5 Alk-, 2 Alk unk), 4 AITL, 1 CTCL with transformation, and 1 T-lymphoblastic lymphoma.
  • Median prior therapies were 3(Allo) and 1(Auto).
  • Median time from diagnosis to Allo or Auto was 18 and 8mo, respectively.
  • For patients with resistant or relapsed disease, Allo should be strongly considered rather than Auto.

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  • (PMID = 27960993.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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22. Bartlett N, Forero-Torres A, Rosenblatt J, Fanale M, Horning SJ, Thompson S, Sievers EL, Kennedy DA: Complete remissions with weekly dosing of SGN-35, a novel antibody-drug conjugate (ADC) targeting CD30, in a phase I dose-escalation study in patients with relapsed or refractory Hodgkin lymphoma (HL) or systemic anaplastic large cell lymphoma (sALCL). J Clin Oncol; 2009 May 20;27(15_suppl):8500

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  • [Title] Complete remissions with weekly dosing of SGN-35, a novel antibody-drug conjugate (ADC) targeting CD30, in a phase I dose-escalation study in patients with relapsed or refractory Hodgkin lymphoma (HL) or systemic anaplastic large cell lymphoma (sALCL).
  • : 8500 Background: A defining feature of HL and sALCL is CD30 expression on malignant cells.
  • The ADC SGN-35 comprises an anti-CD30 antibody conjugated to the antitubulin agent monomethyl auristatin E (MMAE).
  • SGN-35 causes cell cycle arrest and apoptosis by binding to CD30 on the tumor cell surface, internalizing, and releasing MMAE into the cell.
  • Pts with stable disease or better (Cheson 2007) after two 28-day cycles (6 doses) were eligible to continue SGN-35 treatment.

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  • (PMID = 27960851.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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23. Weinberg OK, Seo K, Arber DA: Prevalence of bone marrow involvement in systemic anaplastic large cell lymphoma: are immunohistochemical studies necessary? Hum Pathol; 2008 Sep;39(9):1331-40
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  • [Title] Prevalence of bone marrow involvement in systemic anaplastic large cell lymphoma: are immunohistochemical studies necessary?
  • The frequency of bone marrow involvement in anaplastic large cell lymphoma has been reported with great variation.
  • A prior study found that anaplastic large cell lymphoma involvement of bone marrow was often not evident on routine stains and advocated using immunohistochemical studies.
  • We evaluated 70 bone marrow biopsies from 41 patients with anaplastic large cell lymphoma and found 10 morphologically involved cases (14% of all biopsies, 22% of all patients).
  • In most cases (9/10 biopsies), the involvement of the bone marrow by anaplastic large cell lymphoma was massive and, thus, was evident on the hematoxylin and eosin section.
  • To determine if the hematoxylin and eosin evaluation missed bone marrow involvement, we used a panel of antibodies including CD30, ALK-1, epithelial membrane antigen, and granzyme.
  • Only the 10 morphologically involved cases showed anaplastic large cell lymphoma cells with distinct CD30 expression.
  • Other stains highlighted only a subset of the CD30-positive cases.
  • Overall, marrow involvement in anaplastic large cell lymphoma was relatively uncommon, and when present, it was identified on hematoxylin and eosin sections.
  • [MeSH-major] Bone Marrow / pathology. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Adult. Aged, 80 and over. Antigens, CD30 / immunology. Child. Child, Preschool. Clone Cells / pathology. Eosine Yellowish-(YS). Female. Hematoxylin. Humans. Immunohistochemistry. Male. Middle Aged. Protein-Tyrosine Kinases / analysis. Receptor Protein-Tyrosine Kinases. Survival Analysis

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  • (PMID = 18602674.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase; TDQ283MPCW / Eosine Yellowish-(YS); YKM8PY2Z55 / Hematoxylin
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24. Ries S, Rnjak L, Mitrović Z, Kuvezdić KG, Nola M, Sucić M: CD13+ anaplastic large cell lymphoma with leukemic presentation and additional chromosomal abnormality. Diagn Cytopathol; 2010 Feb;38(2):141-6
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  • [Title] CD13+ anaplastic large cell lymphoma with leukemic presentation and additional chromosomal abnormality.
  • Anaplastic large cell lymphoma (ALCL) is a highly malignant neoplasm characterized by pleomorphic appearance, different immunophenotypes and variable sites of involvement.
  • Expression of myeloid-associated markers in anaplastic large cell lymphomas may mislead the medical team and result in delay of diagnosis due to unusual phenotype.
  • It is important to diagnose this type of tumors and distinguish it from myeloid neoplasms (extramedullary myeloid cell tumors and histiocytic tumors) since therapy and prognosis are significantly different.A 16-year-old female patient presented with fever, lymphadenopathy, and high white blood cell count.
  • Diagnosing a CD13+ ALCL with leukemic presentation with additional cytogenetic abnormality (duplication 5q35) was a significant diagnostic challenge.This combination of features, unusual for lymphoma, should be considered in differential diagnosis of myeloid neoplasms and fatal infections.
  • [MeSH-major] Antigens, CD13 / biosynthesis. Chromosome Aberrations. Lymphoma, Large-Cell, Anaplastic / pathology. Lymphoma, Large-Cell, Anaplastic / physiopathology
  • [MeSH-minor] Adolescent. Cell Separation. Fatal Outcome. Female. Flow Cytometry. Humans. Immunohistochemistry. Immunophenotyping. In Situ Hybridization, Fluorescence

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  • (PMID = 19760763.001).
  • [ISSN] 1097-0339
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 3.4.11.2 / Antigens, CD13
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25. Bakshi NA, Finn WG, Schnitzer B, Valdez R, Ross CW: Fascin expression in diffuse large B-cell lymphoma, anaplastic large cell lymphoma, and classical Hodgkin lymphoma. Arch Pathol Lab Med; 2007 May;131(5):742-7
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  • [Title] Fascin expression in diffuse large B-cell lymphoma, anaplastic large cell lymphoma, and classical Hodgkin lymphoma.
  • Fascin is a sensitive marker for classical Reed-Sternberg cells and has a high negative predictive value for diagnosis of classical Hodgkin lymphoma (CHL).
  • Fascin has been used to distinguish CHL from non-Hodgkin lymphoma.
  • Recently, it was shown that fascin might not help differentiate CHL from anaplastic large cell lymphoma (ALCL).
  • Moreover, fascin has not been extensively studied in the context of other large cell lymphomas.
  • OBJECTIVE: To analyze fascin expression in diffuse large B-cell lymphoma (DLBCL) and also reexamine its usefulness in discriminating CHL from ALCL.
  • DESIGN: Formalin-fixed, paraffin-embedded tissue samples from 41 cases of DLBCL, 30 cases of CHL, and 30 cases of ALCL were analyzed.
  • Fascin expression was compared across each type of lymphoma with additional correlation between fascin positivity and ALK-1 expression in ALCL performed.
  • RESULTS: Only 6 (14.6%) of 41 cases of DLBCL stained positively for fascin, with most neoplastic large cells exhibiting a weak staining pattern.
  • Fifteen (50%) of 30 cases of ALCL showed positivity for fascin, with most large cells staining strongly.
  • All 30 cases of CHL demonstrated intense positive staining.
  • Sixty percent of fascin-positive ALCLs were positive for ALK-1, while 66.7% of fascin-negative ALCLs were positive for ALK-1.
  • CONCLUSIONS: Fascin is highly sensitive for CHL and has a very high negative predictive value (100% in this series) for distinguishing CHL from DLBCL or ALCL.
  • However, the specificity and positive predictive value for fascin are much higher in distinguishing CHL from DLBCL than in distinguishing CHL from ALCL.
  • Expression of fascin appears more useful in the differential diagnosis of CHL versus DLBCL than in the differential diagnosis of CHL versus ALCL.
  • [MeSH-major] Biomarkers, Tumor / analysis. Carrier Proteins / biosynthesis. Hodgkin Disease / metabolism. Lymphoma, B-Cell / metabolism. Lymphoma, Large B-Cell, Diffuse / metabolism. Microfilament Proteins / biosynthesis
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Male. Middle Aged. Predictive Value of Tests. Sensitivity and Specificity


26. Shi YF, Liu CL, Zhou CJ, Gong LP, Dong LN, Li M, Huang X, Gao ZF: [Anaplastic lymphoma kinase gene abnormality and the expression of its fusion protein in primary systemic anaplastic large cell lymphoma]. Beijing Da Xue Xue Bao; 2008 Aug 18;40(4):380-6
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  • [Title] [Anaplastic lymphoma kinase gene abnormality and the expression of its fusion protein in primary systemic anaplastic large cell lymphoma].
  • OBJECTIVE: To study the expression of anaplastic lymphoma kinase (ALK) and chromosome breakage of the anaplastic lymphoma kinase (ALK) gene retrospectively and to investigate their possible value as indicators of prognosis in primary systemic anaplastic large cell lymphomas (S-ALCL).
  • METHODS: Twenty-eight cases of S-ALCL were collected from the Lymphoma Lab, the Department of Pathology, Peking University Health Science Center and Beijing Children's Hospital.
  • The morphologic characteristics were studied under light microscope, and essential immunohistochemical staininings (IHC) were performed and reviewed to confirm the diagnosis of S-ALCL.
  • Locus specific interphase fluorescence in situ hybridization (LSI-FISH) was also performed on the neoplastic cells using paraffin-embedded tissues to detect ALK gene abnormality.
  • In 14 ALCL cases, ALK gene breakage was detected by LSI-FISH, using a dual-color break-apart ALK gene DNA (LSI-ALK) probe.
  • CONCLUSION: IHC detection for ALK fusion protein is important to the diagnosis of S-ALCL.
  • Complex abnormalities of ALK gene exist in S-ALCL cases, and different types of ALK gene might lead to different clinical outcome.
  • [MeSH-major] Chromosome Breakage. Lymphoma, Large-Cell, Anaplastic / genetics. Protein-Tyrosine Kinases / genetics

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  • (PMID = 18677384.001).
  • [ISSN] 1671-167X
  • [Journal-full-title] Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
  • [ISO-abbreviation] Beijing Da Xue Xue Bao
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Recombinant Fusion Proteins; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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27. Kovrigina AM, Probatova NA: [Diagnosis of anaplastic large-cell lymphoma]. Arkh Patol; 2005 Nov-Dec;67(6):7-13
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  • [Title] [Diagnosis of anaplastic large-cell lymphoma].
  • Principles of morphological diagnosis of anaplastic large-cell lymphoma (ALCL) are considered, several its variants are distinguished: classic (32 cases), small-cell (4 cases), rich in granulocytes (2 cases) and lymphohistiocytic (1 case).
  • Morphological similarities between ALCL, classic variants of Hodgkin's lymphoma, variants of diffuse large B-cell lymphoma may require immunohistochemical investigation the criteria of which are suggested.
  • [MeSH-major] Antigens, CD / metabolism. Biomarkers, Tumor / metabolism. Lymphoma, Large-Cell, Anaplastic / diagnosis. Lymphoma, Large-Cell, Anaplastic / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Infant. Male. Middle Aged

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  • (PMID = 16405012.001).
  • [ISSN] 0004-1955
  • [Journal-full-title] Arkhiv patologii
  • [ISO-abbreviation] Arkh. Patol.
  • [Language] rus
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor
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28. Park SJ, Kim S, Lee DH, Jeong YP, Bae Y, Han EM, Huh J, Suh C: Primary systemic anaplastic large cell lymphoma in Korean adults: 11 years' experience at Asan Medical Center. Yonsei Med J; 2008 Aug 30;49(4):601-9
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  • [Title] Primary systemic anaplastic large cell lymphoma in Korean adults: 11 years' experience at Asan Medical Center.
  • PURPOSE: Anaplastic large cell lymphoma (ALCL), a CD30+ T-cell non-Hodgkin's lymphoma, represents only 2-8% of lymphoma overall.
  • Information on the clinical findings of primary systemic ALCL in Korea is limited.
  • Our aims were to report the clinical features and outcomes of primary systemic ALCL.
  • PATIENTS AND METHODS: We retrospectively reviewed the medical records of 36 adult patients diagnosed with primary systemic ALCL at Asan Medical Center from February 1995 through June 2006.
  • Univariate analysis showed that performance status (p = 0.035), international prognostic index (IPI) (p = 0.025), and age-adjusted IPI (p = 0.034) were significant prognostic factors for OS, whereas anaplastic lymphoma kinase (ALK) expression did not affect OS (p = 0.483).
  • CONCLUSION: Our retrospective analysis of Korean primary systemic ALCL patients showed that median OS was 49 months and overall response to CHOP was 91%.
  • [MeSH-major] Hospitals. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Disease-Free Survival. Female. Humans. Korea / epidemiology. Male. Middle Aged. Neoplasm Staging. Protein-Tyrosine Kinases / metabolism. Receptor Protein-Tyrosine Kinases. Survival Rate. Time Factors

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  • (PMID = 18729302.001).
  • [ISSN] 0513-5796
  • [Journal-full-title] Yonsei medical journal
  • [ISO-abbreviation] Yonsei Med. J.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
  • [Other-IDs] NLM/ PMC2615286
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29. Hernandez C, Puangsuvan SN, Peterson A, Robinson JK: Localized perineal cutaneous nodules: a case of recurrent systemic anaplastic large-cell lymphoma. Clin Exp Dermatol; 2009 Dec;34(8):e722-5
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  • [Title] Localized perineal cutaneous nodules: a case of recurrent systemic anaplastic large-cell lymphoma.
  • We report an unusual case of localized cutaneous nodules heralding the recurrence of systemic CD30+ anaplastic large-cell lymphoma (ALCL).
  • A 47-year-old woman developed numerous violaceous nodules in the perineal and upper thigh area 3 years after multimodal treatment and complete remission of primary anaplastic large-cell CD30+ lymphoma.
  • Using immunohistochemical and T-cell gene rearrangement analysis, a recurrence of her anaplastic large-cell lymphoma was diagnosed.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / pathology. Neoplasm Recurrence, Local / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Antigens, CD30 / genetics. Cell Transformation, Neoplastic / genetics. Female. Gene Rearrangement, T-Lymphocyte / genetics. Humans. Middle Aged. Stem Cell Transplantation. Treatment Outcome

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  • (PMID = 20055841.001).
  • [ISSN] 1365-2230
  • [Journal-full-title] Clinical and experimental dermatology
  • [ISO-abbreviation] Clin. Exp. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD30
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30. Mencía-Gutiérrez E, Gutiérrez-Díaz E, Salamanca J, Martínez-González MA: Cutaneous presentation on the eyelid of primary, systemic, CD30+, anaplastic lymphoma kinase (ALK)-negative, anaplastic large-cell lymphoma (ALCL). Int J Dermatol; 2006 Jun;45(6):766-9
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  • [Title] Cutaneous presentation on the eyelid of primary, systemic, CD30+, anaplastic lymphoma kinase (ALK)-negative, anaplastic large-cell lymphoma (ALCL).
  • AIM: To report an unusual case of cutaneous presentation on the eyelid of systemic (or nodal), CD30+, anaplastic large-cell lymphoma (ALCL).
  • RESULTS: The histopathologic and immunohistochemical diagnosis was ALCL, T-cell phenotype, strongly positive for CD43 and CD30, and negative for CD3, anaplastic lymphoma kinase (ALK), and B-cell antigens.
  • CONCLUSIONS: Primary, systemic, CD30+, ALK-negative, ALCL presentations generally have a poor prognosis and tend to occur in older individuals, although the clinical outcome is highly variable and difficult to predict in individual cases.
  • Only three cases of ALCL have been described in the ocular adnexae and none was ALK-negative.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Eyelid Neoplasms / pathology. Lymphoma, Large B-Cell, Diffuse / pathology. Protein-Tyrosine Kinases / analysis
  • [MeSH-minor] Adult. Antigens, CD / analysis. Antigens, CD30 / analysis. Combined Modality Therapy. Humans. Lymphatic Metastasis. Male. Receptor Protein-Tyrosine Kinases. Treatment Outcome

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  • (PMID = 16796648.001).
  • [ISSN] 0011-9059
  • [Journal-full-title] International journal of dermatology
  • [ISO-abbreviation] Int. J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD30; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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31. Querfeld C, Khan I, Mahon B, Nelson BP, Rosen ST, Evens AM: Primary cutaneous and systemic anaplastic large cell lymphoma: clinicopathologic aspects and therapeutic options. Oncology (Williston Park); 2010 Jun;24(7):574-87
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary cutaneous and systemic anaplastic large cell lymphoma: clinicopathologic aspects and therapeutic options.
  • Anaplastic large cell lymphoma (ALCL) is a biologic and clinically heterogenous subtype of T-cell lymphoma.
  • Clinically, ALCL may present as localized (primary) cutaneous disease or widespread systemic disease.
  • These two forms of ALCL are distinct entities with different clinical and biologic features.
  • Both types share similar histology, however, with cohesive sheets of large lymphoid cells expressing the Ki-1 (CD30) molecule.
  • Primary cutaneous ALCL (C-ALCL) is part of the spectrum of CD30+ lymphoproliferative diseases of the skin including lymphomatoid papulosis.
  • Using conservative measures, 5-year disease-free survival rates are > 90%.
  • The systemic ALCL type is an aggressive lymphoma that may secondarily involve the skin, in addition to other extranodal sites.
  • Further, systemic ALCL may be divided based on the expression of anaplastic lymphoma kinase (ALK) protein, which is activated most frequently through the nonrandom t(2;5) chromosome translocation, causing the fusion of the nucleophosmin (NPM) gene located at 5q35 to 2p23 encoding the receptor tyrosine kinase ALK.
  • Systemic ALK+ ALCLs have improved prognosis compared with ALK-negative ALCL, although both subtypes warrant treatment with polychemotherapy.
  • Allogeneic and, to a lesser extent, autologous stem cell transplantation play a role in relapsed disease, while the benefit of upfront transplant is not clearly defined.
  • In addition, a multitude of novel therapeutics are being studied, including anti-CD30 antibodies, histone deacetylase inhibitors, immunomodulatory drugs, proteasome inhibitors, and inhibitors of ALK and its downstream signaling pathways.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / pathology. Lymphoma, Large-Cell, Anaplastic / therapy. Lymphoma, Primary Cutaneous Anaplastic Large Cell / pathology. Lymphoma, Primary Cutaneous Anaplastic Large Cell / therapy. Skin Neoplasms / pathology. Skin Neoplasms / therapy
  • [MeSH-minor] Antigens, CD30 / physiology. Humans. Immunophenotyping

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  • [CommentIn] Oncology (Williston Park). 2010 Jun;24(7):587, 592-3 [20669795.001]
  • [CommentIn] Oncology (Williston Park). 2010 Jun;24(7):594 [20669796.001]
  • (PMID = 20669794.001).
  • [ISSN] 0890-9091
  • [Journal-full-title] Oncology (Williston Park, N.Y.)
  • [ISO-abbreviation] Oncology (Williston Park, N.Y.)
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30
  • [Number-of-references] 118
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32. Armstrong G, Szallasi A, Biegel JA, Shurtleff S, Bilaniuk LT, Womer RB, Choi JK: Early molecular detection of central nervous system relapse in a child with systemic anaplastic large cell lymphoma: case report and review of the literature. Pediatr Blood Cancer; 2005 Apr;44(4):400-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Early molecular detection of central nervous system relapse in a child with systemic anaplastic large cell lymphoma: case report and review of the literature.
  • We report a case of anaplastic large cell lymphoma (ALCL) with central nervous system relapse in an 11-year-old boy.
  • The relapse was suspected on morphologic examination of the cytospin preparations of the cerebrospinal fluid (CSF) with a WBC of 10 cells/microl.
  • The patient developed large intracranial metastases, despite systemic, and intrathecal chemotherapy.
  • This case demonstrates the feasibility of detecting ALCL in paucicellular CSF specimens and suggests that even low CSF involvement can herald massive parenchymal disease.
  • [MeSH-major] Central Nervous System Neoplasms / cerebrospinal fluid. Central Nervous System Neoplasms / pathology. Lymphoma, Large-Cell, Anaplastic / cerebrospinal fluid. Lymphoma, Large-Cell, Anaplastic / pathology

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  • (PMID = 15515044.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 25
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33. Recavarren RA, Yang J: Cytomorphologic features of primary anaplastic large cell lymphoma of the psoas muscle: a case report and literature review. Diagn Cytopathol; 2010 Mar;38(3):208-12
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  • [Title] Cytomorphologic features of primary anaplastic large cell lymphoma of the psoas muscle: a case report and literature review.
  • Ki-1 (CD30) positive anaplastic large cell lymphoma (ALCL) is an uncommon malignancy, which may present with nodal as well as extra-nodal disease.
  • Primary skeletal muscle Ki-1 (CD30) positive ALCL is an even rarer event with few cases reported in the literature and only some with published cytomorphologic features.
  • Smears demonstrated a predominantly discohesive population of large pleomorphic cells.
  • Morphologic and immunohistochemical features later revealed a primary psoas muscle Ki-1 (CD30) positive ALCL with negative staining for anaplastic large cell lymphoma kinase (ALK).
  • Cytologic features of ALCL mimic epithelial neoplasms, sarcomas, melanoma and other lymphomas.
  • Although rare, ALCL should be a diagnostic consideration when discohesive pleomorphic malignant cells are encountered on FNA of a muscle neoplasm.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / pathology. Muscle Neoplasms / pathology. Psoas Muscles / pathology
  • [MeSH-minor] Aged, 80 and over. Antigens, CD30 / metabolism. Biomarkers, Tumor / metabolism. Biopsy, Fine-Needle. Carcinoma / diagnosis. Cell Nucleus / metabolism. Cell Nucleus / pathology. Diagnosis, Differential. Female. Humans. Melanoma / diagnosis. Melanoma / secondary. Protein-Tyrosine Kinases / metabolism. Receptor Protein-Tyrosine Kinases. Skin Neoplasms / diagnosis

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  • (PMID = 19760764.001).
  • [ISSN] 1097-0339
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30; 0 / Biomarkers, Tumor; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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34. Forero-Torres A, Leonard JP, Younes A, Rosenblatt JD, Brice P, Bartlett NL, Bosly A, Pinter-Brown L, Kennedy D, Sievers EL, Gopal AK: A Phase II study of SGN-30 (anti-CD30 mAb) in Hodgkin lymphoma or systemic anaplastic large cell lymphoma. Br J Haematol; 2009 Jul;146(2):171-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A Phase II study of SGN-30 (anti-CD30 mAb) in Hodgkin lymphoma or systemic anaplastic large cell lymphoma.
  • SGN-30, a chimeric anti-CD30 monoclonal antibody, has demonstrated potent preclinical antitumour activity in both Hodgkin lymphoma (HL) and anaplastic large cell lymphoma (ALCL).
  • We conducted an open-label, Phase II study to determine the safety and objective response rate of SGN-30 in 79 patients with refractory/recurrent HL (n = 38) or systemic ALCL (n = 41).
  • Patients had received a median of 3 (range 1-5) prior regimens of chemotherapy or systemic therapy.
  • In the ALCL group, two patients achieved a complete response and five additional patients achieved a partial response, with response durations ranging from 27 to 1460+ d.
  • No objective responses were observed in the HL group; however, 11 patients (29%) had stable disease (duration 62-242 days).
  • Although adverse events were common, most were mild or moderate, and no specific pattern of adverse events was observed in either disease group.
  • These results demonstrate that weekly administration of SGN-30 is safe, with modest clinical activity in patients with ALCL.
  • [MeSH-major] Antibodies, Monoclonal / administration & dosage. Antineoplastic Agents / administration & dosage. Hodgkin Disease / therapy. Lymphoma, Large-Cell, Anaplastic / drug therapy


35. Feldman AL, Law ME, Inwards DJ, Dogan A, McClure RF, Macon WR: PAX5-positive T-cell anaplastic large cell lymphomas associated with extra copies of the PAX5 gene locus. Mod Pathol; 2010 Apr;23(4):593-602

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] PAX5-positive T-cell anaplastic large cell lymphomas associated with extra copies of the PAX5 gene locus.
  • Cell lineage is the major criterion by which lymphomas are classified.
  • Immunohistochemistry has greatly facilitated lymphoma diagnosis by detecting expression of lineage-associated antigens.
  • Anaplastic large cell lymphoma is a T-cell lymphoma that shows morphologic and phenotypic overlap with classical Hodgkin's lymphoma, which is a tumor of B-cell derivation.
  • Staining for the B-cell transcription factor, paired box 5 (PAX5), has been suggested to be helpful in this differential, as it is positive in most classical Hodgkin's lymphomas, but absent in anaplastic large cell lymphomas.
  • In this study we report four systemic T-cell anaplastic large cell lymphomas that were positive for PAX5 by immunohistochemistry, with weak staining intensity similar to that observed in classical Hodgkin's lymphoma.
  • All diagnoses were confirmed by a combination of morphologic, phenotypic, and molecular criteria.
  • Three cases were anaplastic lymphoma kinase (ALK) negative and one was ALK positive.
  • PAX5 immunohistochemistry was negative in 198 additional peripheral T-cell lymphomas, including 66 anaplastic large cell lymphomas.
  • Unexpectedly, although PAX5 translocations were absent, all evaluable PAX5-positive anaplastic large cell lymphomas showed extra copies of the PAX5 gene locus by fluorescence in situ hybridization (FISH).
  • In contrast, only 4% of PAX5-negative peripheral T-cell lymphomas had extra copies of PAX5.
  • We conclude that aberrant expression of PAX5 occurs rarely in T-cell anaplastic large cell lymphomas, and may be associated with extra copies of the PAX5 gene.
  • PAX5-positive lymphomas with morphologic features overlapping different lymphoma types should be evaluated with an extensive immunohistochemical panel and/or molecular studies to avoid diagnostic errors that could lead to inappropriate treatment.
  • As PAX5 overexpression causes T-cell neoplasms in experimental models, PAX5 may have contributed to lymphomagenesis in our cases.

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  • (PMID = 20118907.001).
  • [ISSN] 1530-0285
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA097274-08; United States / NCI NIH HHS / CA / P50 CA097274; United States / NCI NIH HHS / CA / P50 CA097274-08
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / B-Cell-Specific Activator Protein; 0 / PAX5 protein, human
  • [Other-IDs] NLM/ NIHMS167829; NLM/ PMC2848697
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36. Shimoyama Y, Sakakibara A, Kawai K, Nagasaka T, Nakamura S: Molecular diagnosis of malignant lymphoma: mantle cell lymphoma, anaplastic large cell lymphoma, and marginal zone B-cell lymphoma of malt. Nagoya J Med Sci; 2006 Jan;68(1-2):1-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Molecular diagnosis of malignant lymphoma: mantle cell lymphoma, anaplastic large cell lymphoma, and marginal zone B-cell lymphoma of malt.
  • Malignant lymphoma is a heterogeneous category embracing three major types of lymphoid neoplasms: B cell neoplasms, T and NK cell neoplasms, and Hodgkin lymphoma.
  • Within each type, distinct disease entities are defined based on a combination of morphology, immunophenotype, genetic features and clinical syndromes, the emphasis on which represents a new paradigm in the lymphoma classification of the World Health Organization (WHO).
  • These lymphoma entities often have distinctive cytogenetic abnormalities, usually involving translocations that place a potential cellular oncogene under the influence of the immunoglobulin in some low-grade B-cell lymphomas.
  • Both pathologists and oncologists are now concerned with better understanding each disease entity and its spectrum of morphology, genetic events, and clinical behaviors.
  • Over the last decade, significant progress has been made in the molecular characterizations of mantle cell lymphoma, anaplastic large cell lymphoma, and marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), which have not only provided insights into the pathogenesis of lymphomas, but also valuable data that could lead to therapies based on their clinical behavior.
  • [MeSH-major] Biomarkers, Tumor / analysis. Lymphoma, B-Cell / diagnosis. Lymphoma, B-Cell, Marginal Zone / diagnosis. Lymphoma, Large-Cell, Anaplastic / diagnosis. Lymphoma, Mantle-Cell / diagnosis. Molecular Diagnostic Techniques

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  • (PMID = 16579170.001).
  • [ISSN] 0027-7622
  • [Journal-full-title] Nagoya journal of medical science
  • [ISO-abbreviation] Nagoya J Med Sci
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / API2-MALT1 fusion protein, human; 0 / Biomarkers, Tumor; 0 / Oncogene Proteins, Fusion; 136601-57-5 / Cyclin D1; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
  • [Number-of-references] 23
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37. Leong MY, English M, McMullan D, Ramani P: Aberrant expression of beta-HCG in anaplastic large cell lymphoma. Pediatr Dev Pathol; 2008 May-Jun;11(3):230-4
Genetic Alliance. consumer health - Lymphoma, large-cell.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aberrant expression of beta-HCG in anaplastic large cell lymphoma.
  • We report a case of anaplastic large cell lymphoma (ALCL) showing aberrant expression of beta subunit of human chorionic gonadotrophin (beta-HCG).
  • The patient was a 14-year-old boy who presented with a right inguinal mass and a raised serum beta-HCG level.
  • Biopsy of the mass revealed a malignant neoplasm composed of large, pleomorphic cells with prominent nucleoli.
  • These malignant cells showed positive staining with CD30, ALK, epithelial membrane antigen, and beta-HCG.
  • Chromosomal analysis showed t(2;5)(p23;q35) translocation, and polymerase chain reaction demonstrated T-cell receptor gene rearrangement.
  • The patient did not respond well to chemotherapy, and he died 8 months after the diagnosis.
  • To the best of our knowledge, this is the 1st case of ALCL showing aberrant expression of beta-HCG and associated with a raised serum level of beta-HCG.
  • [MeSH-major] Chorionic Gonadotropin, beta Subunit, Human / biosynthesis. Lymphoma, Large-Cell, Anaplastic / metabolism. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Clinical Trials, Phase II as Topic. Diagnosis, Differential. Fatal Outcome. Gene Rearrangement, T-Lymphocyte. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. Neoplasms, Germ Cell and Embryonal / pathology. Polymerase Chain Reaction. Translocation, Genetic

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  • (PMID = 17990918.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chorionic Gonadotropin, beta Subunit, Human
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38. Medeiros LJ, Elenitoba-Johnson KS: Anaplastic Large Cell Lymphoma. Am J Clin Pathol; 2007 May;127(5):707-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Anaplastic Large Cell Lymphoma.
  • Session 8 of the 2005 Society of Hematopathology/European Association for Haematopathology Workshop was devoted to anaplastic large cell lymphoma (ALCL).
  • Most cases submitted were anaplastic lymphoma kinase (ALK)+ ALCL highlighting unusual clinical settings, histologic variants, and variant translocation partners.
  • Cases submitted as ALK- ALCL emphasized the immunohistochemical overlap with classical Hodgkin lymphoma (eg, CD15+/CD30+).
  • It was also clear that consensus histologic and immunohistochemical criteria for the diagnosis of ALK-ALCL are lacking.
  • Many expressed the opinion that ALK-ALCL is not a distinct entity at the immunophenotypic or genetic level and is better designated as peripheral T-cell lymphoma (PTCL), unspecified.
  • Others suggested that the histologic features of ALK-ALCL are distinctive nevertheless and that this diagnosis has meaning that is lost by designating these neoplasms as PTCL, unspecified.
  • This session also included CD30+ anaplastic lymphomas involving skin in which the differential diagnosis included cutaneous ALCL and systemic ALK-ALCL.
  • [MeSH-major] Lymphoma, Large B-Cell, Diffuse / pathology. Protein-Tyrosine Kinases / analysis
  • [MeSH-minor] Antigens, CD30 / analysis. Flow Cytometry. Humans. Immunophenotyping. Receptor Protein-Tyrosine Kinases. Skin Neoplasms / pathology

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  • (PMID = 17511113.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
  • [Number-of-references] 40
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39. Colomba A, Courilleau D, Ramel D, Billadeau DD, Espinos E, Delsol G, Payrastre B, Gaits-Iacovoni F: Activation of Rac1 and the exchange factor Vav3 are involved in NPM-ALK signaling in anaplastic large cell lymphomas. Oncogene; 2008 Apr 24;27(19):2728-36
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Activation of Rac1 and the exchange factor Vav3 are involved in NPM-ALK signaling in anaplastic large cell lymphomas.
  • The majority of anaplastic large cell lymphomas (ALCLs) express the nucleophosmin-anaplastic lymphoma kinase (NPM-ALK) fusion protein, which is oncogenic due to its constitutive tyrosine kinase activity.
  • Transformation by NPM-ALK not only increases proliferation, but also modifies cell shape and motility in both lymphoid and fibroblastic cells.
  • We report that the Rac1 GTPase, a known cytoskeletal regulator, is activated by NPM-ALK in ALCL cell lines (Karpas 299 and Cost) and transfected cells (lymphoid Ba/F3 cells, NIH-3T3 fibroblasts).
  • Moreover, Vav3 is phosphorylated in NPM-ALK positive biopsies from patients suffering from ALCL, demonstrating the pathological relevance of this observation.
  • The use of Vav3-specific shRNA and a dominant negative Rac1 mutant demonstrates the central role of GTPases in NPM-ALK elicited motility and invasion.
  • [MeSH-major] Guanine Nucleotide Exchange Factors / metabolism. Lymphoma, Large-Cell, Anaplastic / enzymology. Protein-Tyrosine Kinases / metabolism. Protein-Tyrosine Kinases / physiology. Proto-Oncogene Proteins c-vav / metabolism. Signal Transduction / physiology. rac1 GTP-Binding Protein / metabolism
  • [MeSH-minor] Animals. Cell Line, Tumor. Humans. Mice. NIH 3T3 Cells. Nuclear Proteins / physiology. Phosphatidylinositol 3-Kinases / physiology. Receptor Protein-Tyrosine Kinases. src-Family Kinases / physiology

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  • (PMID = 17998938.001).
  • [ISSN] 1476-5594
  • [Journal-full-title] Oncogene
  • [ISO-abbreviation] Oncogene
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Guanine Nucleotide Exchange Factors; 0 / Nuclear Proteins; 0 / Proto-Oncogene Proteins c-vav; 0 / VAV3 protein, human; 0 / Vav3 protein, mouse; 117896-08-9 / nucleophosmin; EC 2.7.1.- / Phosphatidylinositol 3-Kinases; EC 2.7.1.- / p80(NPM-ALK) protein; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase; EC 2.7.10.2 / CSK tyrosine-protein kinase; EC 2.7.10.2 / src-Family Kinases; EC 3.6.5.2 / rac1 GTP-Binding Protein
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40. Asha LK, Thomas D, Binitha MP, Nandakumar G: Primary cutaneous multifocal CD30+ anaplastic large cell lymphoma. Indian J Dermatol Venereol Leprol; 2006 Sep-Oct;72(5):376-8
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  • [Title] Primary cutaneous multifocal CD30+ anaplastic large cell lymphoma.
  • Biopsy and immunophenotyping revealed CD 30+ anaplastic primary cutaneous large cell lymphoma.
  • Primary cutaneous anaplastic large cell lymphoma is characterized by single or grouped reddish-brown tumor nodules, which frequently tend to ulcerate.
  • [MeSH-major] Antigens, CD30 / biosynthesis. Lymphoma, Large B-Cell, Diffuse / diagnosis. Lymphoma, Large B-Cell, Diffuse / immunology. Skin Neoplasms / diagnosis. Skin Neoplasms / immunology

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  • (PMID = 17050936.001).
  • [ISSN] 0973-3922
  • [Journal-full-title] Indian journal of dermatology, venereology and leprology
  • [ISO-abbreviation] Indian J Dermatol Venereol Leprol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Antigens, CD30; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; VB0R961HZT / Prednisone; CHOP protocol
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41. Benner MF, Willemze R: Bone marrow examination has limited value in the staging of patients with an anaplastic large cell lymphoma first presenting in the skin. Retrospective analysis of 107 patients. Br J Dermatol; 2008 Nov;159(5):1148-51
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Bone marrow examination has limited value in the staging of patients with an anaplastic large cell lymphoma first presenting in the skin. Retrospective analysis of 107 patients.
  • BACKGROUND: According to criteria of the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas a diagnosis of primary cutaneous CD30-positive anaplastic large cell lymphoma (C-ALCL) should be made only when systemic localizations have been excluded by adequate staging procedures, including a bone marrow biopsy.
  • It has recently been questioned whether or not bone marrow examination should be performed routinely in indolent cutaneous lymphomas such as C-ALCL.
  • OBJECTIVES: To determine the incidence of bone marrow involvement in patients with an ALCL first presenting in the skin to find out if the current policy to advise bone marrow examination should be maintained or whether a bone marrow biopsy should be performed only in selected cases.
  • METHODS: All patients presenting with skin lesions with histological and immunophenotypical features of an ALCL were retrieved from the database of the Dutch Cutaneous Lymphoma Group.
  • Patients with a history of systemic ALCL and patients without bone marrow examination were excluded from the study.
  • The final study group included 107 patients with an ALCL first presenting in the skin, who had been staged completely.
  • RESULTS: Staging procedures showed the presence of extracutaneous disease in 20 patients, but bone marrow involvement was not detected in any of the 107 patients.
  • CONCLUSIONS: Bone marrow examination has limited value in the staging of patients with an ALCL first presenting in the skin, and should be performed only in selected cases.
  • [MeSH-major] Bone Marrow / pathology. Lymphoma, Large-Cell, Anaplastic / pathology. Skin Neoplasms / pathology

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  • (PMID = 18782320.001).
  • [ISSN] 1365-2133
  • [Journal-full-title] The British journal of dermatology
  • [ISO-abbreviation] Br. J. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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42. Yaakup H, Sagap I, Fadilah SA: Primary oesophageal Ki (CD30)-positive ALK+ anaplastic large cell lymphoma of T-cell phenotype. Singapore Med J; 2008 Oct;49(10):e289-92
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  • [Title] Primary oesophageal Ki (CD30)-positive ALK+ anaplastic large cell lymphoma of T-cell phenotype.
  • Primary oesophageal lymphoma is a very rare entity, with fewer than 30 reported cases worldwide.
  • Most of the oesophageal lymphomas are diffuse large B-cell type, with only one reported case of anaplastic large cell lymphoma (ALCL) of T-cell phenotype.
  • Primary oesophageal lymphomas that are not associated with an immunocompromised state tend to affect elderly patients.
  • We describe the first case of primary oesophageal Ki (CD30)-positive ALK+ALCL of T-cell phenotype in a 34-year-old immunocompetent woman, who presented with a two-year history of dysphagia.
  • She was treated with chemotherapy and endoscopic oesophageal dilations and stenting, resulting in complete remission of the lymphoma and resolution of the dysphagia.
  • She then underwent autologous peripheral blood haematopoietic stem cell transplantation and remained disease-free two years after the diagnosis.
  • [MeSH-major] Antigens, CD30 / biosynthesis. Esophageal Neoplasms / metabolism. Esophageal Neoplasms / pathology. Lymphoma, Large-Cell, Anaplastic / metabolism. Lymphoma, Large-Cell, Anaplastic / pathology. Protein-Tyrosine Kinases / biosynthesis
  • [MeSH-minor] Adult. Disease-Free Survival. Endoscopy / methods. Female. Hematopoietic Stem Cell Transplantation / methods. Humans. Phenotype. Receptor Protein-Tyrosine Kinases. Remission Induction. T-Lymphocytes / metabolism. Tomography, X-Ray Computed / methods. Treatment Outcome

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  • (PMID = 18946602.001).
  • [ISSN] 0037-5675
  • [Journal-full-title] Singapore medical journal
  • [ISO-abbreviation] Singapore Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Singapore
  • [Chemical-registry-number] 0 / Antigens, CD30; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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43. Sugiyama H, Asagoe K, Morizane S, Oono T, Okazaki F, Iwatsuki K: Leukocyte common antigen-negative, aggressive cutaneous anaplastic large cell lymphoma with prominent pseudocarcinomatous hyperplasia. Eur J Dermatol; 2008 Jan-Feb;18(1):74-7
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  • [Title] Leukocyte common antigen-negative, aggressive cutaneous anaplastic large cell lymphoma with prominent pseudocarcinomatous hyperplasia.
  • Patients with anaplastic large cell lymphoma (ALCL) often present with tumor-mediated skin changes, including pseudocarcinomatous hyperplasia (PCH), acquired ichthyosis, and tissue neutrophilia.
  • We report a 58-year-old male patient with leukocyte common antigen (LCA)-negative, null cell-type ALCL associated with marked PCH mimicking undifferentiated squamous cell carcinoma.
  • Although lymphocyte markers were lacking, the CD30 expression and the clonal rearrangement of the T-cell receptor gamma gene confirmed the diagnosis of ALCL.
  • The patient had an aggressive clinical course, in which the tumor cells metastasized to the regional lymph nodes a few months after surgical removal of the primary lesion, and skin nodules recurred on the face despite intensive polychemotherapy, followed by autologous peripheral blood stem cell transplantation.
  • The diagnosis of ALCL was delayed in our case because of the prominent PCH, the lack of LCA, and the unusually rapid progression of the tumor.
  • [MeSH-major] Antigens, CD45 / analysis. Lymphoma, Large-Cell, Anaplastic / pathology. Nose Neoplasms / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Carcinoma, Squamous Cell / diagnosis. Diagnosis, Differential. Humans. Hyperplasia. Lymphatic Metastasis. Male. Middle Aged


44. Rodig SJ, Ouyang J, Juszczynski P, Currie T, Law K, Neuberg DS, Rabinovich GA, Shipp MA, Kutok JL: AP1-dependent galectin-1 expression delineates classical hodgkin and anaplastic large cell lymphomas from other lymphoid malignancies with shared molecular features. Clin Cancer Res; 2008 Jun 1;14(11):3338-44
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  • [Title] AP1-dependent galectin-1 expression delineates classical hodgkin and anaplastic large cell lymphomas from other lymphoid malignancies with shared molecular features.
  • We recently found that Reed-Sternberg cell Gal1 promotes the immunosuppressive T-helper 2/T-regulatory cell-skewed microenvironment in classical Hodgkin lymphoma (cHL).
  • In addition, because there are common signaling and survival pathways in cHL and additional non-Hodgkin lymphomas, we also evaluated whether the AP1/Gal1 signature is shared by other molecularly or morphologically related lymphomas.
  • EXPERIMENTAL DESIGN: We evaluated 225 cases of primary cHL and non-Hodgkin lymphoma for evidence of a functional AP1/Gal1 signature by immunohistochemical techniques.
  • In contrast, diffuse large B-cell lymphoma, primary mediastinal large B-cell lymphoma, and another Hodgkin-related entity, nodular lymphocyte-predominant Hodgkin lymphoma, do not express Gal1.
  • However, anaplastic large cell lymphoma (ALCL), consistently expresses both Gal1 and its transcriptional regulator, c-Jun.
  • The presence of activated c-Jun, indicative of functional AP1 activity, was confirmed by phospho-c-Jun immunostaining in cHL and ALCL.
  • CONCLUSIONS: These findings establish a functional AP1 signature that includes Gal1 expression in cHL and ALCL and suggests a common mechanism for tumor immunotolerance in these diseases.
  • In addition, the combination of Gal1 and c-Jun serve as diagnostic biomarkers that delineate cHL and ALCL from other lymphomas with shared morphologic and/or molecular features.
  • [MeSH-major] Biomarkers, Tumor / analysis. Galectin 1 / biosynthesis. Hodgkin Disease / metabolism. Lymphoma, Large-Cell, Anaplastic / metabolism. Lymphoproliferative Disorders / metabolism. Transcription Factor AP-1 / metabolism
  • [MeSH-minor] Diagnosis, Differential. Gene Expression. Humans. Immunohistochemistry. Proto-Oncogene Proteins c-jun / metabolism. Reed-Sternberg Cells / metabolism

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  • (PMID = 18519761.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Galectin 1; 0 / Proto-Oncogene Proteins c-jun; 0 / Transcription Factor AP-1
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45. Chuang SS, Hsieh YC, Ye H, Hwang WS: Lymphohistiocytic anaplastic large cell lymphoma involving skin: a diagnostic challenge. Pathol Res Pract; 2009;205(4):283-7
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  • [Title] Lymphohistiocytic anaplastic large cell lymphoma involving skin: a diagnostic challenge.
  • Systemic anaplastic large cell lymphoma (ALCL) involving the skin should be differentiated from primary cutaneous CD30-positive T-cell lymphoproliferative disorders.
  • The lymphohistiocytic variant of ALCL (LH-ALCL) is rich in reactive histiocytes with relatively few neoplastic cells, which pose a diagnostic challenge.
  • We present a case of LH-ALCL involving skin mimicking granulomatous inflammation.
  • Biopsy of the cervical lymph node showed LH-ALCL with null cell phenotype.
  • Microscopically, the cutaneous lesion was located predominately around the hair follicle, with numerous reactive histiocytes and scanty medium-sized lymphoma cells expressing CD30 and anaplastic lymphoma kinase (ALK) protein.
  • Furthermore, an ALK gene rearrangement was demonstrated by locus-specific interphase fluorescent in situ hybridization, confirming cutaneous involvement with LH-ALCL.
  • LH-ALCL involving the skin is a rare event, and the numerous reactive histiocytes may mask scanty tumor cells.
  • In addition to B-and T-cell markers, (dermato) pathologists must be aware of this entity in cutaneous lymphohistiocytic proliferations and perform immunostaining for CD30 and ALK to reach a correct diagnosis.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Adult. Antigens, CD30 / metabolism. Gene Rearrangement. Histiocytes / pathology. Humans. In Situ Hybridization, Fluorescence. Male. Protein-Tyrosine Kinases / genetics. Protein-Tyrosine Kinases / metabolism. Receptor Protein-Tyrosine Kinases

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  • (PMID = 19091487.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antigens, CD30; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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46. Miranda RN, Lin L, Talwalkar SS, Manning JT, Medeiros LJ: Anaplastic large cell lymphoma involving the breast: a clinicopathologic study of 6 cases and review of the literature. Arch Pathol Lab Med; 2009 Sep;133(9):1383-90
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  • [Title] Anaplastic large cell lymphoma involving the breast: a clinicopathologic study of 6 cases and review of the literature.
  • CONTEXT: Lymphomas involving the breast are rare, and most cases are of B-cell lineage; T-cell neoplasms represent less than 10% of all breast lymphomas.
  • OBJECTIVE: To define the clinicopathologic spectrum of anaplastic large cell lymphomas (ALCLs) involving the breast.
  • DESIGN: Six cases of ALCL involving the breast were identified at a single institution during 21 years.
  • There were 4 anaplastic lymphoma kinase- negative (ALK(-)) ALCL cases; 3 of these neoplasms developed around breast implants.
  • Two patients with ALK(-) ALCL had a history of cutaneous ALCL.
  • There were 2 ALK(+) ALCLs; both patients had stage IV disease.
  • Histologically, all neoplasms were composed of large anaplastic cells that were uniformly CD30(+) and expressed markers of T-cell lineage.
  • Four patients with adequate follow-up are alive, with a mean of 4.1 years (range, 1.5-9 years) after diagnosis of the breast tumor.
  • Included in this group are 2 patients with ALK(-) ALCL associated with breast implants who were alive 4 years and 9 years after diagnosis.
  • CONCLUSIONS: Including the 6 cases we describe, a total of 21 cases of ALCL involving the breast are reported.
  • Patients with cutaneous ALCL can subsequently develop ALK(-) ALCL involving the breast, and these tumors can be associated with breast implants.
  • [MeSH-major] Breast Neoplasms / pathology. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Adult. Aged. Antigens, CD30 / metabolism. Biomarkers, Tumor / metabolism. Breast Implantation. Combined Modality Therapy. DNA, Neoplasm / analysis. Fatal Outcome. Female. Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / genetics. Humans. Middle Aged. Protein-Tyrosine Kinases / metabolism. Receptor Protein-Tyrosine Kinases. Young Adult

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  • (PMID = 19722744.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30; 0 / Biomarkers, Tumor; 0 / DNA, Neoplasm; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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47. Passoni L, Gallo B, Biganzoli E, Stefanoni R, Massimino M, Di Nicola M, Gianni AM, Gambacorti-Passerini C: In vivo T-cell immune response against anaplastic lymphoma kinase in patients with anaplastic large cell lymphomas. Haematologica; 2006 Jan;91(1):48-55
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  • [Title] In vivo T-cell immune response against anaplastic lymphoma kinase in patients with anaplastic large cell lymphomas.
  • BACKGROUND AND OBJECTIVES: Anaplastic lymphoma kinase (ALK) oncogenic fusion proteins, expressed in about 60% of anaplastic large cell lymphomas (ALCL), are tumor-specific molecular targets for such a malignancy.
  • DESIGN AND METHODS: The frequency and the functional phenotype of the anti-ALK CD8 precursor repertoire in freshly isolated peripheral blood mononuclear cells (PBMC) from healthy donors and ALK-positive patients were determined by major histocompatibility complex (MHC)/tetrameric analyses.
  • In addition, the ability of the anti-ALK immune response to specifically lyse ALK-positive lymphoma cells was investigated by in vitro stimulation with ALK-derived peptide p280-89.
  • RESULTS: Tetrameric MHC/peptide complexes revealed high frequencies of CD8/ALK-tetramer-positive cells both in patients and in healthy individuals.
  • However, the functional phenotype of the CD8/ALK-tetramer-positive lymphocytes showed the presence of effector and memory T lymphocytes only in patients.
  • A polyclonal ALK-specific tumor-reactive T-cell line was isolated from patients' peripheral blood lymphocytes.
  • INTERPRETATION AND CONCLUSIONS: The presence of an anti-ALK effector/memory lymphocyte population in the peripheral blood of ALK-positive patients indicates an in vivo antigenic challenge.
  • Thus, ALK is a lymphoma-associated antigen suitable for immune interventions.
  • The high number of anti-ALK memory CD8 T cells present in patients' PBMC may represent a valid source of activated CTL suitable for cancer cell lysis.
  • [MeSH-major] CD8-Positive T-Lymphocytes / immunology. Lymphoma, Large-Cell, Anaplastic / immunology. Protein-Tyrosine Kinases / immunology

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  • (PMID = 16434370.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Cancer Vaccines; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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48. Muzzafar T, Wei EX, Lin P, Medeiros LJ, Jorgensen JL: Flow cytometric immunophenotyping of anaplastic large cell lymphoma. Arch Pathol Lab Med; 2009 Jan;133(1):49-56
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  • [Title] Flow cytometric immunophenotyping of anaplastic large cell lymphoma.
  • CONTEXT: Anaplastic large cell lymphoma (ALCL) is usually diagnosed by histologic and immunohistochemical analysis.
  • OBJECTIVE: To review our experience using flow-cytometric immunophenotyping to assess cases of ALCL and to evaluate the diagnostic utility of this technique.
  • DESIGN: Each case of ALCL was assessed by flow cytometry with 3-color or 4-color antibody panels, and data were reanalyzed by cluster analysis using Paint-a-Gate for cases with retrievable flow cytometry data files.
  • Anaplastic lymphoma kinase (ALK) was assessed by using immunohistochemistry.
  • RESULTS: Twenty-three ALCL cases were analyzed by flow cytometry.
  • In the remaining 19 cases (11 ALK(+), 8 ALK(-)), all were positive for CD30 and CD45.
  • Anaplastic large cell lymphoma cells were large and usually CD45 bright, with many or most cells falling in the region of monocytes on the CD45/side scatter plot.
  • The frequencies of T-cell antigen expression in ALK(+) cases were CD2, 67%; CD7, 60%; CD3, 45%; CD4, 33%; CD5, 14%; and CD8, 14%.
  • In ALK(-) cases, the frequencies of the T-cell antigen expression were CD2, 100%; CD3, 50%; CD4, 40%; CD7, 40%; CD5, 25%; and CD8, 20%.
  • CONCLUSIONS: Flow cytometry can be used to immunophenotype ALCL cases.
  • [MeSH-major] Flow Cytometry / methods. Immunophenotyping / methods. Lymphoma, Large-Cell, Anaplastic / diagnosis
  • [MeSH-minor] Adult. Aged. Antigens, CD / metabolism. Biopsy, Fine-Needle. Female. Humans. Immunohistochemistry. Male. Middle Aged. Protein-Tyrosine Kinases / metabolism. Receptor Protein-Tyrosine Kinases

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  • (PMID = 19123736.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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49. Piva R, Pellegrino E, Inghirami G: Identification and validation of the anaplastic large cell lymphoma signature. Adv Exp Med Biol; 2007;604:129-36
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  • [Title] Identification and validation of the anaplastic large cell lymphoma signature.
  • Anaplastic large cell lymphomas (ALCL) represent a subset of lymphomas in which the anaplastic lymphoma kinase (ALK) gene is fused to several partners, most frequently to the NPM gene.
  • We have previously demonstrated that the constitutive expression and phosphorylation of ALK chimeric proteins is sufficient for cellular transformation, and its activity is strictly required for the survival of ALCL cells.
  • To unravel signaling pathways required for NPM-ALK-mediated transformation and tumor maintenance, we analyzed the transcriptomes of ALK positive ALCL cell lines through experimentally controlled approaches in which ALK signaling was abrogated by an inducible ALKshRNA or by ALK inhibitors.
  • A functional RNAi screening identified new ALK transcriptional targets instrumental to cell transformation and/or to sustain the growth and survival of ALK positive ALCL cells.
  • [MeSH-major] Gene Expression Regulation. Gene Expression Regulation, Neoplastic. Lymphoma, Large B-Cell, Diffuse / diagnosis. Lymphoma, Large B-Cell, Diffuse / genetics

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  • (PMID = 17695725.001).
  • [ISSN] 0065-2598
  • [Journal-full-title] Advances in experimental medicine and biology
  • [ISO-abbreviation] Adv. Exp. Med. Biol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01-CA64033
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / RNA, Messenger
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50. Kolonić SO, Prasek-Kudrna K, Roso V, Radić-Kristo D, Planinc-Peraica A, Dzebro S, Kardum-Skelin I, Jaksić B: Value of fine-needle aspiration cytology in diagnosis of Hodgkin's lymphoma and anaplastic large cell lymphoma: one centre experience. Coll Antropol; 2010 Mar;34(1):75-9
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  • [Title] Value of fine-needle aspiration cytology in diagnosis of Hodgkin's lymphoma and anaplastic large cell lymphoma: one centre experience.
  • The aim of the study was to determine the value and limitations of cytology in diagnosis of Hodgkin's lymphoma (HL) and anaplastic large cell lymphoma (ALCL) as well as differentiation between these two entities.
  • We analysed the FNA cytodiagnoses and histopathological reports, as well as treatment and survival in 89 newly diagnosed consecutive patients with these lymphomas treated in our clinical department.
  • Cytology revealed 65 classic HL, 18 ALCL and three patients in which diagnosis was not informative.
  • Among 65 FNA cytodiagnoses of HL, comparison with histopathology was made in 61 cases and the histopathological diagnoses were as follows: 56 (91.8%) HL; three ALCL; one diffuse large B cell lymphoma and one marginal zone B cell lymphoma.
  • In the group of 18 FNA cytodiagnoses of ALCL eight patients (53.3%) had definitive diagnosis of ALCL (either as T-cell or O type), five (33.3%) of HL and in three cases a histopathological diagnosis could not be made.
  • These results confirm the value of FNA in diagnostic procedure in patients with HL and ALCL, especially in HL group of patients.
  • Since we have an almost uniform group of patients according to therapeutic approach, we did univariate analyses and found out that patients with FNA cytodiagnoses of HL, younger than 55 years, with early stage of the disease and without B symptoms had significantly longer overall survival (OS).
  • FNA cytodiagnosis has clinical relevance in differentiation between HL and ALCL.
  • [MeSH-major] Biopsy, Fine-Needle / standards. Hodgkin Disease / mortality. Hodgkin Disease / pathology. Lymphoma, Large-Cell, Anaplastic / mortality. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Female. Humans. Lymphoma, Large B-Cell, Diffuse / mortality. Lymphoma, Large B-Cell, Diffuse / pathology. Male. Middle Aged. Reproducibility of Results. Survival Analysis. Young Adult

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  • (PMID = 20432736.001).
  • [ISSN] 0350-6134
  • [Journal-full-title] Collegium antropologicum
  • [ISO-abbreviation] Coll Antropol
  • [Language] eng
  • [Publication-type] Journal Article; Validation Studies
  • [Publication-country] Croatia
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51. Belloni-Fortina A, Montesco MC, Piaserico S, Bordignon M, Tona F, Feltrin G, Alaibac M: Primary cutaneous CD30+ anaplastic large cell lymphoma in a heart transplant patient: case report and literature review. Acta Derm Venereol; 2009;89(1):74-7
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  • [Title] Primary cutaneous CD30+ anaplastic large cell lymphoma in a heart transplant patient: case report and literature review.
  • The risk of a post-transplant lymphoproliferative disorder is 28-49 times the risk of a lymphoproliferative disorder in the normal population.
  • Most cases are of B-cell phenotype and are associated with Epstein-Barr virus infection.
  • Post-transplant lymphoproliferative disorders presenting clinically in the skin are rare and usually of B-cell phenotype.
  • Only rare cases of cutaneous T-cell post-transplant lymphoproliferative disorder have been reported previously, mostly mycosis fungoides type.
  • We describe here a rare primary cutaneous T-cell lymphoma CD30+ arising in a heart transplant patient who had a nodule on the right leg, several years after heart transplantation.
  • The morphology and immunohistochemical findings were consistent with a CD30+ anaplastic large cell lymphoma with a T-cell phenotype.
  • In this patient, the presence of a solitary lesion and the lack of systemic involvement represented the main factors taken into account in choosing the therapy and the patient was therefore treated using a non-aggressive approach, although with systemic immunosuppression.
  • In conclusion, the diagnosis of a CD30+ anaplastic large cell lymphoma in transplant recipients does not imply aggressive clinical behaviour by the lymphoma.
  • [MeSH-major] Antigens, CD30 / analysis. Heart Transplantation. Lymphoma, T-Cell, Cutaneous / etiology. Skin Neoplasms / etiology


52. Mosunjac MB, Sundstrom JB, Mosunjac MI: Unusual presentation of anaplastic large cell lymphoma with clinical course mimicking fever of unknown origin and sepsis: autopsy study of five cases. Croat Med J; 2008 Oct;49(5):660-8
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  • [Title] Unusual presentation of anaplastic large cell lymphoma with clinical course mimicking fever of unknown origin and sepsis: autopsy study of five cases.
  • AIM: To describe a subset of cases with the unusual clinical and histomorphological presentation of anaplastic large cell lymphoma (ALCL) mimicking fever of unknown origin (FUO) and sepsis.
  • METHODS: A pathology database was searched using full term Systematized Nomenclature of Medicine codes for ALCL to identify 23ALCL cases from the period 1999-2006.
  • Of those, five cases that did not have a correct premortem diagnosis were further analyzed to elucidate the reasons for delayed and incorrect pre-mortem diagnosis.
  • The analyzed data included clinical presentation, duration of symptoms, duration of hospital stay, premortem presumed cause of death, white blood cell count, platelet count, anion gap and blood pH, liver enzymes (alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, alkaline phosphatase), lactate, coagulation tests (prothrombin time, partial thromboplastin time, fibrinogen, D-dimers), microbiology cultures, and radiology and surgical pathology reports.
  • Autopsy reports were reviewed for description of major gross findings, initial clinical diagnosis, and cause of death.
  • RESULTS: Five fatal and pre-mortem unrecognized ALCL cases were characterized by rapid decline, with histologic findings showing predominantly extranodal involvement, intravascular lymphomatosis, and hemophagocytosis.
  • CONCLUSIONS: There is a distinct group of ALCLs with unique and specific clinical, gross autopsy, and histopathologic findings.
  • Recognition of this clinical variant may facilitate early detection and potentially timely diagnosis and therapy.
  • [MeSH-major] Fever of Unknown Origin / etiology. Lymphoma, Large-Cell, Anaplastic / complications. Lymphoma, Large-Cell, Anaplastic / diagnosis. Sepsis / etiology

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  • (PMID = 18925700.001).
  • [ISSN] 1332-8166
  • [Journal-full-title] Croatian medical journal
  • [ISO-abbreviation] Croat. Med. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Croatia
  • [Other-IDs] NLM/ PMC2582359
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53. Siordia-Reyes AG, Ferman-Cano F, Rodríguez-Velasco A: [ALK-1 positive anaplastic large cell lymphoma of the lung. Report of a pediatric case]. Gac Med Mex; 2005 Nov-Dec;141(6):531-4
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  • [Title] [ALK-1 positive anaplastic large cell lymphoma of the lung. Report of a pediatric case].
  • [Transliterated title] Linfoma anaplásico de células grandes ALK-1 positivo, primario de pulmón. Informe de un caso pediátrico.
  • Ki-1 anaplastic large cell non-Hodgkin lymphoma is a well recognized clinical entity.
  • In the extranodal disease, which occurs in approximately half of the cases, the skin is the most common site; bone marrow, lung and central nervous system are generally not involved.
  • Anaplastic large cell lymphoma is more common among young people, in whom the prognosis is more favorable.
  • Primary anaplastic large cell lymphoma of the lung is a rare clinical entity.
  • Its clinical expression is similar to a high grade malignant lymphoma, and in most cases the diagnosis is made in advanced stages.
  • We present a pediatric case with ALK-1 positive anaplastic large cell lymphoma of the lung.
  • [MeSH-major] Lung Neoplasms / chemistry. Lymphoma, Large B-Cell, Diffuse / chemistry. Membrane Proteins / analysis
  • [MeSH-minor] Activin Receptors, Type II. Child. Humans. Male

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  • (PMID = 16381509.001).
  • [ISSN] 0016-3813
  • [Journal-full-title] Gaceta médica de México
  • [ISO-abbreviation] Gac Med Mex
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Mexico
  • [Chemical-registry-number] 0 / Membrane Proteins; EC 2.7.11.30 / ACVRL1 protein, human; EC 2.7.11.30 / Activin Receptors, Type II
  • [Number-of-references] 16
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54. Engsig FN, Møller MB, Hasselbalch HK, Mahdi B, Obel N: Extreme neutrophil granulocytosis in a patient with anaplastic large cell lymphoma of T-cell lineage. APMIS; 2007 Jun;115(6):778-83
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  • [Title] Extreme neutrophil granulocytosis in a patient with anaplastic large cell lymphoma of T-cell lineage.
  • All microbiology tests and test for autoimmune disease were negative.
  • Conventional pathological examination of bone marrow and lymph node biopsies did not demonstrate malignant cells and inflammatory disease was suspected.
  • Immunohistochemistry and cytogenetics postmortem led to a diagnosis of anaplastic large cell lymphoma (ALCL) of T-cell lineage.
  • Involvement of peripheral blood with leukemoid reaction is a rare manifestation of ALCL.
  • [MeSH-major] Granulocytes / pathology. Lymphoma, Large-Cell, Anaplastic / pathology. Neutrophils / pathology

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  • (PMID = 17550390.001).
  • [ISSN] 0903-4641
  • [Journal-full-title] APMIS : acta pathologica, microbiologica, et immunologica Scandinavica
  • [ISO-abbreviation] APMIS
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
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55. Gustafson S, Medeiros LJ, Kalhor N, Bueso-Ramos CE: Anaplastic large cell lymphoma: another entity in the differential diagnosis of small round blue cell tumors. Ann Diagn Pathol; 2009 Dec;13(6):413-27
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  • [Title] Anaplastic large cell lymphoma: another entity in the differential diagnosis of small round blue cell tumors.
  • We saw in consultation a biopsy specimen from a 6-year old girl with anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL).
  • Immunohistochemical workup with a large panel of antibodies at another institution showed immunoreactivity for NB84 and neuron specific enolase (dim).
  • Antibodies specific for CD3, CD20, and CD45/LCA were negative; CD30 or ALK were not assessed.
  • At the time of our review, we considered the possibility of ALCL.
  • Immunohistochemical analysis for CD30 showed bright, uniform expression and ALK was positive in a nuclear and cytoplasmic pattern, confirming the diagnosis of ALK+ ALCL.
  • The purpose of this review is to discuss ALK+ ALCL and many of the other entities included under the rubric of small round blue cell tumor, with a focus on tumors that occur in children.
  • [MeSH-major] Lymphoma / pathology. Lymphoma, Large-Cell, Anaplastic / pathology. Neoplasms, Complex and Mixed / pathology. Neuroblastoma / pathology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology. Rhabdomyosarcoma / pathology. Sarcoma, Ewing / pathology
  • [MeSH-minor] Biomarkers, Tumor / metabolism. Child. Diagnosis, Differential. Disease-Free Survival. Female. Humans. Immunohistochemistry. Protein-Tyrosine Kinases / metabolism. Receptor Protein-Tyrosine Kinases


56. Skiljevic D, Nikolic MM, Milinkovic M, Bonaci-Nikolic B: Regressing anaplastic CD30-positive large-cell lymphoma of the skin. Acta Dermatovenerol Alp Pannonica Adriat; 2006 Sep;15(3):131-4
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  • [Title] Regressing anaplastic CD30-positive large-cell lymphoma of the skin.
  • Originally believed to be of histiocytic origin, regressing primary cutaneous anaplastic large-cell lymphoma is a CD30 (Ki 1) positive T-cell lymphoma with histologic high grade malignancy, but with an often favorable clinical course with regression of individual lesions.
  • The clinical, histologic, and immunohistochemical findings pointed to CD30 positive primary cutaneous anaplastic large-cell lymphoma.
  • After 2 years of activity, the disease regressed.
  • During the 10-year follow-up period, no signs of disease reactivation were noted.
  • Accurate recognition of this lymphoma is important to avoid unnecessary aggressive treatments.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / diagnosis. Skin Neoplasms / diagnosis
  • [MeSH-minor] Aged. Diagnosis, Differential. Female. Humans. Leg. Remission, Spontaneous

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  • (PMID = 17053848.001).
  • [ISSN] 1318-4458
  • [Journal-full-title] Acta dermatovenerologica Alpina, Pannonica, et Adriatica
  • [ISO-abbreviation] Acta Dermatovenerol Alp Pannonica Adriat
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Slovenia
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57. Gudgin E, Rashbass J, Pulford KJ, Erber WN: Primary and isolated anaplastic large cell lymphoma of the bone marrow. Leuk Lymphoma; 2005 Mar;46(3):461-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary and isolated anaplastic large cell lymphoma of the bone marrow.
  • Anaplastic large cell lymphoma (ALCL) is a T-cell lymphoma in which the majority of patients present with advanced stage III or IV disease.
  • Here we report a case of ALCL where bone marrow was the only site of disease, in a 60-year-old man with pyrexia and pancytopenia.
  • The diagnosis of ALCL was made on detection of CD30-positive anaplastic cells in the bone marrow, together with prominent hemophagocytosis.
  • Genetics confirmed the clonal nature of the disease and showed it to be anaplastic lymphoma kinase (ALK) negative.
  • Primary isolated bone marrow ALCL should be considered in the diagnosis of pancytopenia associated with hemophagocytosis.
  • [MeSH-major] Bone Marrow / pathology. Lymphoma, Large-Cell, Anaplastic / pathology

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  • (PMID = 15621840.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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58. Buxton D, Bacchi CE, Gualco G, Weiss LM, Zuppan CW, Rowsell EH, Huang Q, Wang J: Frequent expression of CD99 in anaplastic large cell lymphoma: a clinicopathologic and immunohistochemical study of 160 cases. Am J Clin Pathol; 2009 Apr;131(4):574-9
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  • [Title] Frequent expression of CD99 in anaplastic large cell lymphoma: a clinicopathologic and immunohistochemical study of 160 cases.
  • By using conventional paraffin immunoperoxidase staining and tissue microarrays, we retrospectively investigated CD99 expression in a series of 160 anaplastic large cell lymphoma (ALCL) cases.
  • Of the 160 cases, 103 (64.4%) were positive for CD99.
  • CD99 expression was present in 96 (64.4%) of 149 of the common type, 4 (80%) of 5 of the small cell variant, and 3 (50%) of 6 of the lymphohistiocytic variant cases.
  • CD99 expression was slightly more frequent in anaplastic large cell lymphoma kinase (ALK)+ cases compared with ALK- cases (43/54 [80%] vs 44/81 [54%]).
  • With 2 exceptions, ALK+ ALCL was seen only in patients younger than 41 years.
  • We conclude that CD99 is frequently expressed in ALCL, with a slightly increased frequency in the younger age ALK+ cases.
  • Nodal and extranodal ALCL should be considered in the differential diagnosis when a CD99+ neoplasm is encountered.
  • [MeSH-major] Antigens, CD / biosynthesis. Biomarkers, Tumor / analysis. Cell Adhesion Molecules / biosynthesis. Lymphoma, Large-Cell, Anaplastic / metabolism

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  • (PMID = 19289593.001).
  • [ISSN] 1943-7722
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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59. Savage KJ, Harris NL, Vose JM, Ullrich F, Jaffe ES, Connors JM, Rimsza L, Pileri SA, Chhanabhai M, Gascoyne RD, Armitage JO, Weisenburger DD, International Peripheral T-Cell Lymphoma Project: ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project. Blood; 2008 Jun 15;111(12):5496-504
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  • [Title] ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project.
  • The International Peripheral T-Cell Lymphoma Project is a collaborative effort designed to gain better understanding of peripheral T-cell and natural killer (NK)/T-cell lymphomas (PTCLs).
  • Of the 1314 eligible patients, 181 had anaplastic large-cell lymphoma (ALCL; 13.8%) on consensus review: One hundred fifty-nine had systemic ALCL (12.1%) and 22 had primary cutaneous ALCL (1.7%).
  • Patients with anaplastic lymphoma kinase-positive (ALK(+)) ALCL had a superior outcome compared with those with ALK(-) ALCL (5-year failure-free survival [FFS], 60% vs 36%; P = .015; 5-year overall survival [OS], 70% vs 49%; P = .016).
  • However, contrary to prior reports, the 5-year FFS (36% vs 20%; P = .012) and OS (49% vs 32%; P = .032) were superior for ALK(-) ALCL compared with PTCL, not otherwise specified (PTCL-NOS).
  • Patients with primary cutaneous ALCL had a very favorable 5-year OS (90%), but with a propensity to relapse (5-year FFS, 55%).
  • In summary, ALK(-) ALCL should continue to be separated from both ALK(+) ALCL and PTCL-NOS.
  • Although the prognosis of ALK(-) ALCL appears to be better than that for PTCL-NOS, it is still unsatisfactory and better therapies are needed.
  • Primary cutaneous ALCL is associated with an indolent course.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / mortality. Lymphoma, Large-Cell, Anaplastic / pathology. Lymphoma, T-Cell, Peripheral / mortality. Lymphoma, T-Cell, Peripheral / pathology. Protein-Tyrosine Kinases / immunology
  • [MeSH-minor] Adult. Asia. Diagnosis, Differential. Europe. Female. Follow-Up Studies. Humans. Immunophenotyping. International Cooperation. Male. Middle Aged. North America. Prognosis. Receptor Protein-Tyrosine Kinases. Recurrence. Retrospective Studies. Skin Neoplasms / immunology. Skin Neoplasms / mortality. Skin Neoplasms / pathology. Skin Neoplasms / therapy. Survival Analysis. Treatment Outcome

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  • (PMID = 18385450.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
  • [Investigator] Savage K; Connors J; Gascoyne R; Chhanabhai M; Wilson W; Jaffe E; Armitage J; Vose J; Weisenburger D; Anderson J; Ullrich F; Bast M; Hochberg E; Harris N; Levine A; Nathwani B; Miller T; Rimsza L; Montserrat E; Lopez-Guillermo A; Campo E; Cuadros M; Alvarez Ferreira J; Martinez Delgado B; Holte H; Delabie J; Rüdiger T; Müller-Hermelink K; Reimer P; Adam P; Wilhelm M; Schmitz N; Nerl C; Lister A; Norton A; MacLennan KA; Zinzani PL; Pileri S; Federico M; Bellei M; Coiffier B; Berger F; Tanin I; Wannakrairot P; Au W; Liang R; Loong F; Rajan S; Sng I; Tobinai K; Matsuno Y; Morishima Y; Nakamura S; Seto M; Tanimoto M; Yoshino T; Suzumiya J; Ohshima K; Kim WS; Ko YH
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60. Takahashi D, Nagatoshi Y, Nagayama J, Inagaki J, Itonoaga N, Takeshita M, Okamura J: Anaplastic large cell lymphoma in leukemic presentation: a case report and a review of the literature. J Pediatr Hematol Oncol; 2008 Sep;30(9):696-700
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  • [Title] Anaplastic large cell lymphoma in leukemic presentation: a case report and a review of the literature.
  • It is extremely rare that a patient with anaplastic large cell lymphoma (ALCL) demonstrates circulating lymphoma cells.
  • The white cell count was 26.2x10(9)/L with 95% of abnormal lymphoid cells, which were small to medium-sized with a high nucleus/cytoplasm ratio, basophilic cytoplasm, condensed nuclear chromatins, and 1 or 2 distinct nucleoli, hemoglobin 6.4 g/dL, and platelet 0.9x10(9)/L.
  • A flow cytometric analysis of abnormal cells in both the peripheral blood and bone marrow samples was strongly positive for CD30 on their cell membranes.
  • Reverse transcriptase-polymerase chain reaction of peripheral blood cell-derived mRNA also indicated the fusion gene product of anaplastic lymphoma kinase and nucleophosmin.
  • Subsequently, the patient was diagnosed to have ALCL with a rare clinical feature of a peripheral leukemic presentation, and his disease revealed to be refractory to chemotherapy.
  • On the basis of the 11 childhood cases of ALCL with leukemic presentation so far published and reviewed herein, the prognosis is very poor.
  • [MeSH-major] Leukemia / diagnosis. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Antigens, CD30 / analysis. Bone Marrow Examination. Child. Fatal Outcome. Humans. Male. Protein-Tyrosine Kinases. Translocation, Genetic

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  • (PMID = 18776764.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30; EC 2.7.1.- / p80(NPM-ALK) protein; EC 2.7.10.1 / Protein-Tyrosine Kinases
  • [Number-of-references] 25
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61. Gorgidze LA, Vorob'ev IA: [A morphometric analysis of the nuclei and nucleoli in tumor cells in lymphogranulomatosis, diffuse large B-cell lymphoma and anaplastic large cell lymphoma]. Ter Arkh; 2009;81(2):71-5
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  • [Title] [A morphometric analysis of the nuclei and nucleoli in tumor cells in lymphogranulomatosis, diffuse large B-cell lymphoma and anaplastic large cell lymphoma].
  • AIM: To make a comparative morphometric analysis of the nuclei and nucleoli of tumor cells in lymphogranulomatosis (LGM), diffuse large B-cell lymphoma (DLBCL) and anaplastic large cell lymphoma (ALCL) for differential diagnosis of these lymphomas.
  • RESULTS: Mean area of tumor cell nuclei in LGM was 97.25 +/- 68.77 mcm2, in DLBCL and ALCL--55.89 +/- 20.13 mcm2 and 70.31 +/- 34.64 mcm2, respectively.
  • Hodgkin's and Berezovsky-Rid-Sternberg cell bucleoli area was the largest (11.44 +/- 7.83 mcm2).
  • Mean area of the nucleoli in DLBCL was 3.05 +/- 1.58, in ALCL--5.53 +/- 4.94 mcm2.
  • CONCLUSION: Nucleoli in Hodgkin 's cells are significantly larger than those in the tumor cells in ALCL and DLBCL and the nucleoli with the area more than 12 mcm2 can be used in differential diagnosis between LGM and DLBCL but not between LGM and ALCL.
  • [MeSH-major] Cell Nucleolus / pathology. Cell Nucleus / pathology. Hodgkin Disease / pathology. Lymphoma, Large B-Cell, Diffuse / pathology. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Biopsy. Diagnosis, Differential. Humans


62. Splinter A, Min S, Balkian A, Quinn JJ: Pediatric anaplastic large cell lymphoma presenting as generalized lymphadenopathy. Oncology (Williston Park); 2010 Sep;24(10):954-6, 958
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  • [Title] Pediatric anaplastic large cell lymphoma presenting as generalized lymphadenopathy.
  • Because a finding of generalized lymphadenopathy can be associated with such a wide range of diseases and conditions, determining its cause can sometimes be challenging.
  • Ultimately, however, anaplastic large cell lymphoma (ALCL) was diagnosed.
  • Using this case as a backdrop, we discuss the wide range of systemic illnesses that the differential diagnosis of generalized lymphadenopathy encompasses--including infectious, autoimmune, and oncological disorders.
  • Finally, we present an overview of ALCL, one of the more rare pediatric malignancies.
  • [MeSH-major] Lymphatic Diseases / diagnosis. Lymphoma, Large-Cell, Anaplastic / diagnosis
  • [MeSH-minor] Child, Preschool. Diagnosis, Differential. Female. Humans

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  • (PMID = 21138178.001).
  • [ISSN] 0890-9091
  • [Journal-full-title] Oncology (Williston Park, N.Y.)
  • [ISO-abbreviation] Oncology (Williston Park, N.Y.)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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63. Ardigò M, Marulli GC, Cota C, Mastroianni A, Berardesca E: Bexarotene and interferon-alpha combination therapy in a patient affected by relapsing anaplastic large cell lymphoma with cutaneous involvement. J Drugs Dermatol; 2007 Feb;6(2):216-9
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  • [Title] Bexarotene and interferon-alpha combination therapy in a patient affected by relapsing anaplastic large cell lymphoma with cutaneous involvement.
  • A 74-year-old man presented with relapsing systemic anaplastic large cell lymphoma (ALCL) with cutaneous involvement who had a third recurrence of cutaneous lesions associated with inguinal lymphonodes enlargement.
  • Two months after stopping therapy, lymphonodal relapse of the lymphoma was seen; however, cutaneous lesions were still in complete remission.
  • Association of low dose bexarotene with interferon-xalphaseems to represent a possible alternative therapy for relapsing systemic ALCL presenting as prevalent cutaneous involvement in patients with severe worsening of general conditions.
  • In our case, this protocol was unable to maintain a longer disease free survival in comparison with the 2 previous polychemotherapy cycles.
  • Further extended studies are required in order to define the possible rule of this combination therapy in relapsing systemic ALCL.
  • [MeSH-major] Interferon-alpha / therapeutic use. Lymphoma, Large B-Cell, Diffuse / drug therapy. Skin Neoplasms / drug therapy. Tetrahydronaphthalenes / therapeutic use
  • [MeSH-minor] Aged. Disease-Free Survival. Drug Therapy, Combination. Humans. Lymph Nodes / pathology. Male. Recurrence. Remission Induction / methods. Salvage Therapy / methods

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  • (PMID = 17373182.001).
  • [ISSN] 1545-9616
  • [Journal-full-title] Journal of drugs in dermatology : JDD
  • [ISO-abbreviation] J Drugs Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Interferon-alpha; 0 / Tetrahydronaphthalenes; A61RXM4375 / bexarotene
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64. Yang CS, Chou G, Jan YJ, Wang J, Yeh DC, Teng CL: Primary lymphohistiocytic variant of anaplastic large cell lymphoma of the stomach. J Chin Med Assoc; 2007 Feb;70(2):71-5
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  • [Title] Primary lymphohistiocytic variant of anaplastic large cell lymphoma of the stomach.
  • Here, we report an unusual case of gastric anaplastic large cell lymphoma (ALCL), lymphohistiocytic variant, in a 70-year-old female patient who presented with epigastric pain, tarry stool and body weight loss.
  • Endoscopic and imaging findings revealed a Bormann type II tumor in the stomach with perigastric lymphadenopathy and multiple tumor nodules in the liver.
  • Histologically, both gastric and hepatic tumors demonstrated anaplastic large neoplastic cells scattered among numerous reactive histiocytes.
  • Immunostaining of these tumor cells reacted positively for CD30, CD3, CD45 RO/UCHL1, and negatively for epithelial membrane antigen, CD68, lysozyme, CD15, CD79a, CD138, PAX5 and anaplastic lymphoma kinase.
  • Both the morphologic and immunophenotypic findings supported the diagnosis of gastric ALCL of lymphohistiocytic variant with liver metastasis.
  • This patient then received chemotherapy and was still alive after 17 months of follow-up, without evidence of residual disease.
  • [MeSH-major] Histiocytes / pathology. Lymphoma, Large B-Cell, Diffuse / pathology. Stomach Neoplasms / pathology


65. Anastasov N, Bonzheim I, Rudelius M, Klier M, Dau T, Angermeier D, Duyster J, Pittaluga S, Fend F, Raffeld M, Quintanilla-Martinez L: C/EBPβ expression in ALK-positive anaplastic large cell lymphomas is required for cell proliferation and is induced by the STAT3 signaling pathway. Haematologica; 2010 May;95(5):760-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] C/EBPβ expression in ALK-positive anaplastic large cell lymphomas is required for cell proliferation and is induced by the STAT3 signaling pathway.
  • BACKGROUND: Anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma is characterized by the t(2;5) chromosomal translocation, resulting in the expression of a fusion protein formed of nucleophosmin (NPM) and ALK.
  • Recently, we reported the abnormal expression of the transcription factor CCAAT/enhancer binding protein-beta (C/EBPbeta) in ALK-positive anaplastic large cell lymphomas, and demonstrated its dependence on NPM-ALK activity.
  • DESIGN AND METHODS: In this study, the role of C/EBPbeta in proliferation and survival of ALK-positive anaplastic large cell lymphomas was investigated, as well as the mechanism of its expression and activity.
  • RESULTS: Interference with C/EBPbeta expression resulted in a dramatic decrease in cell proliferation in ALK-positive anaplastic large cell lymphomas, with a mild induction of apoptosis after 6 days.
  • Down-regulation of STAT3 resulted in a marked decrease in C/EBPbeta mRNA and protein levels with impairment in cell proliferation and viability, underscoring the important role of these two proteins in ALK-mediated oncogenesis.
  • [MeSH-major] CCAAT-Enhancer-Binding Protein-beta / biosynthesis. Cell Proliferation. Gene Expression Regulation, Neoplastic. Lymphoma, Large-Cell, Anaplastic / enzymology. Receptor Protein-Tyrosine Kinases / biosynthesis. STAT3 Transcription Factor / physiology. Signal Transduction
  • [MeSH-minor] Animals. Cell Line. Cell Line, Transformed. Cell Line, Tumor. Cell Survival / genetics. Down-Regulation / genetics. Humans. Mice


66. Damm-Welk C, Busch K, Burkhardt B, Schieferstein J, Viehmann S, Oschlies I, Klapper W, Zimmermann M, Harbott J, Reiter A, Woessmann W: Prognostic significance of circulating tumor cells in bone marrow or peripheral blood as detected by qualitative and quantitative PCR in pediatric NPM-ALK-positive anaplastic large-cell lymphoma. Blood; 2007 Jul 15;110(2):670-7
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  • [Title] Prognostic significance of circulating tumor cells in bone marrow or peripheral blood as detected by qualitative and quantitative PCR in pediatric NPM-ALK-positive anaplastic large-cell lymphoma.
  • Clinical and histopathological characteristics have limited prognostic value for children with anaplastic large-cell lymphoma (ALCL).
  • We evaluated the presence, extent, and prognostic impact of circulating tumor cells in bone marrow (BM) and peripheral blood (PB) of children and adolescents with NPM-ALK-positive ALCL at diagnosis using qualitative and quantitative polymerase chain reaction (PCR) for NPM-ALK.
  • BM was positive for NPM-ALK in 47.5% of patients, and positivity was significantly correlated with clinical stage, mediastinal or visceral involvement, microscopic BM involvement, and histologic subtype.
  • BM PCR was associated with the cumulative incidence of relapses (CI-Rs): CI-R was 50% +/- 10% for 38 PCR-positive and 15% +/- 7% for 42 PCR-negative patients (P < .001).
  • [MeSH-major] Bone Marrow Cells / physiology. Lymphoma, Large B-Cell, Diffuse / genetics. Protein-Tyrosine Kinases / genetics

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  • (PMID = 17392503.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA Primers; EC 2.7.1.- / p80(NPM-ALK) protein; EC 2.7.10.1 / Protein-Tyrosine Kinases
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67. Asano N, Suzuki R, Matsuo K, Kagami Y, Ishida F, Tamaru JI, Jin GS, Sato Y, Shimoyama Y, Yoshino T, Morishima Y, Nakamura S: Cytotoxic molecule expression is predictive of prognosis in Hodgkin's-like anaplastic large cell lymphoma. Histopathology; 2007 May;50(6):705-15
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  • [Title] Cytotoxic molecule expression is predictive of prognosis in Hodgkin's-like anaplastic large cell lymphoma.
  • AIMS: The Revised European American Lymphoma classification uses the term Hodgkin's-like anaplastic large cell lymphoma (HD-like ALCL) for borderline cases with features of both anaplastic large cell lymphoma (ALCL) and classical Hodgkin's lymphoma (HL).
  • The aim of this study was to clarify the association between cytotoxic molecule (CM) expression and clinical outcome in HD-like ALCL.
  • METHODS AND RESULTS: Subjects were 59 patients with HD-like ALCL, defined by nodal presentation without mediastinal bulky lesions, T- or null-cell phenotype, CD30+ anaplastic lymphoma kinase (ALK)- phenotype and by confluent sheets or nodules of large cells mimicking classic Hodgkin and Reed-Sternberg cells.
  • We evaluated the presenting features and prognosis of subjects on categorization into two defined groups, namely CM (TIA1 and/or granzyme B)-positive (n = 21) and CM-negative (n = 38).
  • The CM+ group had poorer disease-specific survival than the CM- group (P = 0.02) despite the absence of differences in other clinical characteristics.
  • Multivariate analysis confirmed that CM expression was an independent prognostic factor, in contrast to phenotypic categorization (T-cell vs. null-cell group), which had no prognostic impact on disease-specific survival.
  • CONCLUSION: CM expression is predictive of prognosis in HD-like ALCL.
  • [MeSH-major] Granzymes / metabolism. Lymphoma, Large B-Cell, Diffuse / diagnosis. Lymphoma, Large B-Cell, Diffuse / metabolism. Poly(A)-Binding Proteins / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Female. Gene Expression Regulation, Neoplastic. Hodgkin Disease / pathology. Humans. Lymphocytes, Null / pathology. Male. Middle Aged. Phenotype. Predictive Value of Tests. Prognosis. Reed-Sternberg Cells / pathology. Survival Analysis. T-Lymphocytes / pathology

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  • [ErratumIn] Histopathology. 2007 Jun;50(7):962
  • (PMID = 17493234.001).
  • [ISSN] 0309-0167
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Poly(A)-Binding Proteins; 0 / TIA1 protein, human; EC 3.4.21.- / Granzymes
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68. Liu A, Sugisaki Y, Hosone M, Namimatsu S, Maeda S, Naito Z, Ghazizadeh M: CD30-positive diffuse large B-cell lymphoma with microvillous features: so-called microvillous lymphoma. J Clin Pathol; 2009 Sep;62(9):840-4
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  • [Title] CD30-positive diffuse large B-cell lymphoma with microvillous features: so-called microvillous lymphoma.
  • A case of CD30-positive microvillous lymphoma (MVL) in an 87-year-old man who was encountered generalised lymphadenopathy is presented.
  • Histopathologically, the tumour showed a morphological mimic of anaplastic large cell lymphoma (ALCL) with sinusoidal growth pattern.
  • Immunohistochemically (IHC), the tumour cells were CD30(+), CD20(+), CD45(+), BCL-2(+), BCL-6(+), MUM1(+), Ki-67(+), CD45RO(-), CD3(-), CD10(-), CD15(-), CD56(-), EMA(-), TIA-1(-) and ALK(-).
  • Electron microscopically, the tumour cells were similar to large transformed lymphocytes and had circumferentially profuse microvillous projections resembling those of epithelial mesothelioma cells.
  • In conclusion, CD30-positive MVLs are indistinguishable from ALCLs that have ultrastructural microvillous projections by morphology alone.
  • However, the lack of EMA, TIA-1 and ALK expression in this MVL case facilitated a definite distinction from ALCLs.
  • The results of a panel of three markers (CD10(-), Bcl-6(+) and MUM1(+)) suggested that the present case of CD30-positive MVLs has an activated non-germinal centre B-cell origin.
  • [MeSH-major] Antigens, CD30 / analysis. Lymphoma, Large B-Cell, Diffuse / ultrastructure
  • [MeSH-minor] Aged, 80 and over. Biomarkers, Tumor / analysis. Diagnosis, Differential. Humans. Lymphoma, Large-Cell, Anaplastic / diagnosis. Male. Microscopy, Electron. Microvilli / ultrastructure

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  • (PMID = 19126565.001).
  • [ISSN] 1472-4146
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD30; 0 / Biomarkers, Tumor
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69. Kameoka J, Ichinohasama R, Inoue H, Yamamoto J, Yokoyama H, Tomiya Y, Yamada M, Ishizawa K, Harigae H, Sawai T, Sasaki T: CD26, together with cell surface adenosine deaminase, is selectively expressed on ALK-positive, but not on ALK-negative, anaplastic large cell lymphoma and Hodgkin's lymphoma. Leuk Lymphoma; 2006 Oct;47(10):2181-8
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  • [Title] CD26, together with cell surface adenosine deaminase, is selectively expressed on ALK-positive, but not on ALK-negative, anaplastic large cell lymphoma and Hodgkin's lymphoma.
  • CD26/dipeptidyl peptidase IV is a cell surface antigen with multiple biological functions.
  • Although its involvement in tumor biology has been suggested, the significance of its expression in malignant lymphoma has not been clarified in detail.
  • This study examined the expression of CD26 and cell surface adenosine deaminase (ADA) in 42 cases of Hodgkin's lymphoma (HL) and T-cell lymphoma by immunohistochemistry on frozen sections.
  • CD26 was expressed in three of 14 cases of HL, in four of eight cases of anaplastic large cell lymphoma (ALCL), in two of nine cases of peripheral T-cell lymphoma, in one of six cases of lymphoblastic lymphoma and in none of three cases of adult T-cell lymphoma/leukemia.
  • Expression of cell surface ADA was fully correlated with the expression of CD26 and expression of CD26/ADA in ALCL and HL was also completely correlated with the expression of p80 and epithelial membrane antigen.
  • Of 10 CD26-positive patients, seven had fever and elevated CRP at initial diagnosis and over a median follow-up of 61 months (range, 7 - 152 months) only three survived.
  • This study suggested that CD26 is selectively expressed on ALK-positive, but not on ALK-negative, ALCL and HL.
  • This is also the first report to demonstrate that ADA is coexpressed with CD26 on the cell surface of malignant neoplasms in vivo.
  • [MeSH-major] Adenosine Deaminase / biosynthesis. Cell Membrane / enzymology. Dipeptidyl Peptidase 4 / biosynthesis. Gene Expression Regulation, Enzymologic. Gene Expression Regulation, Neoplastic. Hodgkin Disease / metabolism. Lymphoma, Large B-Cell, Diffuse / metabolism. Protein-Tyrosine Kinases / biosynthesis

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  • (PMID = 17071493.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD3; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase; EC 3.4.14.5 / Dipeptidyl Peptidase 4; EC 3.5.4.4 / Adenosine Deaminase
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70. Rupani A, Modi C, Desai S, Rege J: Primary anaplastic large cell lymphoma of central nervous system--a case report. J Postgrad Med; 2005 Oct-Dec;51(4):326-7
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  • [Title] Primary anaplastic large cell lymphoma of central nervous system--a case report.
  • Central nervous system (CNS) involvement is extremely rare in anaplastic large cell lymphoma (ALCL).
  • Primary ALCL of CNS on radiology is often misdiagnosed as tuberculosis.
  • We report a fatal case of primary ALCL of CNS in a 17 year old male.
  • Biopsy showed large pleomorphic cells.
  • Immunohistochemical stains showed positivity for CD30, CD43, EMA and ALK-1.
  • Hence a high level of suspicion is essential for early diagnosis and for instituting appropriate treatment.
  • [MeSH-major] Central Nervous System Neoplasms / diagnosis. Lymphoma, Large B-Cell, Diffuse / diagnosis
  • [MeSH-minor] Adolescent. Diagnostic Errors. Fatal Outcome. Humans. Magnetic Resonance Imaging. Male. Tuberculosis / diagnosis


71. Balachandran I, Walker JW Jr, Broman J: Fine needle aspiration cytology of ALK1(-), CD30+ anaplastic large cell lymphoma post renal transplantation: a case report and literature review. Diagn Cytopathol; 2010 Mar;38(3):213-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Fine needle aspiration cytology of ALK1(-), CD30+ anaplastic large cell lymphoma post renal transplantation: a case report and literature review.
  • Epstein-Barr virus (EBV) related non-Hodgkin's lymphomas of B-cell type is more common than those of T-cell origin.
  • CD30 positive Anaplastic Large Cell Lymphoma (ALCL) is a Non-Hodgkin's lymphoma (B or T cell type) that accounts for a small percentage of PTLD's.
  • ALCL of T-cell type are a spectrum of disease ranging from primary cutaneous to systemic nodal ALCL.
  • The systemic nodal ALCL is further subdivided into anaplastic lymphoma kinase-1 (ALK-1) positive or negative.
  • We present an unusual manifestation of ALK-1 negative CD30 positive ALCL in a post renal transplant patient in FNA cytology with all supportive adjuvant studies and differential diagnoses and review the cytology literature on this topic.
  • [MeSH-major] Antigens, CD30 / analysis. Kidney Transplantation / adverse effects. Lymphoma, Large-Cell, Anaplastic / pathology. Mediastinal Neoplasms / pathology. Protein-Tyrosine Kinases / analysis
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / analysis. Biopsy, Fine-Needle. Carcinoma / diagnosis. Cyclophosphamide / therapeutic use. Diagnosis, Differential. Doxorubicin / therapeutic use. Humans. Immunocompromised Host. Immunohistochemistry. Male. Melanoma / diagnosis. Postoperative Complications. Prednisone / therapeutic use. Receptor Protein-Tyrosine Kinases. Remission Induction. Seminoma / diagnosis. Seminoma / secondary. Testicular Neoplasms / diagnosis. Vincristine / therapeutic use

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  • (PMID = 19774614.001).
  • [ISSN] 1097-0339
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30; 0 / Biomarkers, Tumor; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase; VB0R961HZT / Prednisone; CHOP protocol
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72. Rekhi B, Sridhar E, Viswanathan S, Shet TM, Jambhekar NA: ALK+ anaplastic large cell lymphoma with cohesive, perivascular arrangements on cytology, mimicking a soft tissue sarcoma: a report of 2 cases. Acta Cytol; 2010 Jan-Feb;54(1):75-8
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  • [Title] ALK+ anaplastic large cell lymphoma with cohesive, perivascular arrangements on cytology, mimicking a soft tissue sarcoma: a report of 2 cases.
  • BACKGROUND: An accurate recognition of a lymphoma at an extranodal site is essential to avoid unnecessary excisions.
  • Fine needle aspiration cytology (FNAC) has been recognized as a useful tool in the primary diagnosis of soft tissue tumors.
  • An anaplastic large cell lymphoma (ALCL), occurring in soft tissues, poses a diagnostic challenge.
  • We present the cytomorphology of 2 cases of anaplastic lymphoma kinase (ALK)+ ALCL that displayed a perivascular arrangement, thereby mimicking a sarcoma.
  • Differential diagnoses included a rhabdomyosarcoma and a lymphoma.
  • On biopsy and immunohistochemistry, tumor cells were positive for vimentin, LCA, EMA, CD30 and ALK.
  • CONCLUSION: ALCL should be considered in the differential diagnosis of pediatric soft tissue tumors, especially in cases with multifocal involvement.
  • [MeSH-major] Biopsy, Fine-Needle. Lymphoma, Large-Cell, Anaplastic / metabolism. Lymphoma, Large-Cell, Anaplastic / pathology. Protein-Tyrosine Kinases / metabolism. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Child. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Male. Receptor Protein-Tyrosine Kinases. Sarcoma / pathology

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  • (PMID = 20306994.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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73. Lones MA, Heerema NA, Le Beau MM, Perkins SL, Kadin ME, Kjeldsberg CR, Sposto R, Meadows A, Siegel S, Buckley J, Finlay J, Abromowitch M, Cairo MS, Sanger WG: Complex secondary chromosome abnormalities in advanced stage anaplastic large cell lymphoma of children and adolescents: a report from CCG-E08. Cancer Genet Cytogenet; 2006 Dec;171(2):89-96
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Complex secondary chromosome abnormalities in advanced stage anaplastic large cell lymphoma of children and adolescents: a report from CCG-E08.
  • Among pediatric non-Hodgkin lymphomas, one of the most distinctive types is anaplastic large cell lymphoma (ALCL).
  • Specific chromosomal abnormalities are associated with prognosis in childhood acute lymphoblastic leukemia, but chromosome abnormalities have not been evaluated for prognostic value in pediatric ALCL.
  • For Children's Cancer Group protocol CCG-E-08 Etiologic Study of Non-Hodgkin Lymphoma in Childhood, three patients were enrolled with cytogenetic analysis of ALCL and simultaneously enrolled on treatment protocol CCG-552.
  • Pathology material and karyotypes at initial diagnosis underwent central review.
  • Demographics included ages of 9, 12, and 14 years, and a male/female ratio of 1:2.
  • All patients had advanced disease (stage III).
  • Disease progressed or relapsed in two patients, and one died.
  • In two patients with progressive disease or relapse, additional chromosomal abnormalities at 1q21 and 10q24, possibly involving MCL1 and HOX11/TCL3, respectively, may have contributed to worse outcome.
  • Pediatric ALCL cases frequently have complex karyotypes and usually involve ALK/NPM translocations in this limited study.
  • Additional chromosome abnormalities may be involved in the pathogenesis of ALCL.
  • Further studies are warranted in larger cohorts of children and adolescents with ALCL.
  • [MeSH-major] Chromosome Aberrations. Lymphoma, Large B-Cell, Diffuse / genetics. Lymphoma, Large B-Cell, Diffuse / pathology

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  • (PMID = 17116485.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 13539; United States / NCI NIH HHS / CA / CA 98543
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
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74. Ezra N, Van Dyke GS, Binder SW: CD30 positive anaplastic large-cell lymphoma mimicking Langerhans cell histiocytosis. J Cutan Pathol; 2010 Jul;37(7):787-92
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  • [Title] CD30 positive anaplastic large-cell lymphoma mimicking Langerhans cell histiocytosis.
  • The presence of CD 1a+ dendritic cells (DC) has been well described in T-cell lymphoproliferative disorders, and the presence of large numbers of DCs has rarely been reported as a mimicker of Langerhans cell histiocytsis (LCH).
  • We present the case of a 56-year-old female with a solitary nodule on the chin whose case was referred to our institution for confirmation of the diagnosis of LCH.
  • Skin biopsy showed an ulcerated nodule containing a wedge-shaped infiltrate comprised of large atypical cells and cells with prominent grooved nuclei.
  • The constellation of histologic and immunologic features favored a CD30 lymphoproliferative disorder of T-cell lineage even though there were accompanying numerous dendritic histiocytes and CD1a positive Langerhans cells.
  • The sheets of CD30 positive atypical lymphoid cells which express T-cell markers were consistent with CD30 positive lymphoproliferative disease and favor CD30 positive anaplastic large-cell lymphoma (ALCL) over Langerhans histiocytosis.
  • The absence of Anaplastic Lymphoma Kinase (ALK) staining favored a primary cutaneous origin.
  • This case signifies a CD 30+ ALCL of the skin which histopathologically mimics a LCH.
  • CD30 positive anaplastic large-cell lymphoma (ALCL) mimicking Langerhans cell histiocytosis (LCH).
  • [MeSH-major] Antigens, CD30 / biosynthesis. Histiocytosis, Langerhans-Cell / pathology. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Biomarkers, Tumor / analysis. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Middle Aged


75. Yang HB, Li J, Shen T: Primary anaplastic large cell lymphoma of the lung. Report of two cases and literature review. Acta Haematol; 2007;118(3):188-91
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  • [Title] Primary anaplastic large cell lymphoma of the lung. Report of two cases and literature review.
  • To our knowledge, only eleven cases of primary anaplastic large cell lymphoma (ALCL) of the lung have previously been reported.
  • We describe here another two cases of primary pulmonary ALCL that developed in two Chinese women.
  • A comprehensive workup failed to show disease outside the chest.
  • CD30-positive ALCL was demonstrated by histopathological studies of the lung tissue.
  • Primary pulmonary lymphoma is a great challenge for pneumologists since the clinical presentations and radiological findings are nonspecific.
  • Appropriate invasive biopsy is necessary for early diagnosis.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Lung Neoplasms / drug therapy. Lung Neoplasms / pathology. Lymphoma, Large B-Cell, Diffuse / drug therapy. Lymphoma, Large B-Cell, Diffuse / pathology
  • [MeSH-minor] Adolescent. Adult. Antigens, CD30 / metabolism. Asian Continental Ancestry Group. Biopsy. Cyclophosphamide / administration & dosage. Doxorubicin / administration & dosage. Female. Humans. Prednisone / administration & dosage. Remission Induction. Vincristine / administration & dosage

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  • [Copyright] 2007 S. Karger AG, Basel
  • (PMID = 17934256.001).
  • [ISSN] 1421-9662
  • [Journal-full-title] Acta haematologica
  • [ISO-abbreviation] Acta Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antigens, CD30; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; VB0R961HZT / Prednisone; CHOP protocol
  • [Number-of-references] 15
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76. Tamiolakis D, Papadopoulos N, Venizelos J, Kakagia D, Nikolaidou S, Bolioti S, Kouskoukis C: ALK-positive neutrophil-rich variant of anaplastic large cell lymphoma diagnosed after head trauma. Onkologie; 2005 Jun;28(6-7):356-8
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  • [Title] ALK-positive neutrophil-rich variant of anaplastic large cell lymphoma diagnosed after head trauma.
  • BACKGROUND: Anaplastic lymphoma kinase (ALK) expression has not been described in neutrophil-rich anaplastic large cell lymphoma (NR-ALCL).
  • CASE REPORT: A 12-year old female with a 4-weeks history of a non-resolving bump over the forehead resulting from injury, was diagnosed of stage IE cutaneous T-cell lymphoma, and radiation was employed.
  • Shortly after completion of therapy, there was progress of the disease on the soft tissue of the right hand, and bone marrow involvement was also found.
  • A fine-needle aspiration of the hand mass was performed, and a diagnosis of CD30+/ALK+ NR-ALCL, was rendered.
  • METHODS: We studied the morphological characteristics of CD30+/ALK+ NR-ALCL using histological methods.
  • A panel of antibodies were used to establish diagnosis and subtyping.
  • CONCLUSIONS: ALK-ALCL arising in the skin represents a single disease with a broad spectrum of morphology; clinicians and pathologists should be aware of this neutrophil-rich (NR) variant with aggressive clinical presentation.
  • [MeSH-major] Lymphoma, Large B-Cell, Diffuse / blood. Lymphoma, Large B-Cell, Diffuse / pathology. Neutrophils / pathology. Protein-Tyrosine Kinases / blood. Skin Neoplasms / blood. Skin Neoplasms / pathology

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  • (PMID = 15933425.001).
  • [ISSN] 0378-584X
  • [Journal-full-title] Onkologie
  • [ISO-abbreviation] Onkologie
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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77. Chen RF, Chen CT, Liao HT, Chen CH, Chen YR: Primary cutaneous anaplastic large cell lymphoma of the face presenting as posttraumatic maxillary sinusitis. J Craniofac Surg; 2008 Nov;19(6):1597-9
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  • [Title] Primary cutaneous anaplastic large cell lymphoma of the face presenting as posttraumatic maxillary sinusitis.
  • Primary cutaneous anaplastic large cell lymphoma (ALCL) is an uncommon disease.
  • It has various presentations that may mislead the diagnosis and cause delay of treatment.
  • The patient was first treated for maxillary sinusitis according to clinical history, but the pathology turned out to be primary cutaneous ALCL.
  • The presentations of primary cutaneous ALCL of the face may mimic posttraumatic maxillary sinusitis.
  • [MeSH-major] Cheek / pathology. Facial Neoplasms / diagnosis. Lymphoma, Large-Cell, Anaplastic / diagnosis. Skin Neoplasms / diagnosis
  • [MeSH-minor] Child. Diagnosis, Differential. Facial Injuries / diagnosis. Follow-Up Studies. Humans. Male. Maxillary Fractures / diagnosis. Maxillary Sinusitis / diagnosis. Soft Tissue Infections / diagnosis. Wounds, Nonpenetrating / diagnosis. Zygomatic Fractures / diagnosis

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  • (PMID = 19098559.001).
  • [ISSN] 1536-3732
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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78. Cağlar K, Akyüz C, Uner A, Kutluk T, Yalçin B, Varan A, Büyükpamukçu M: Anaplastic large cell lymphoma in a child presenting with cutaneous nodules and blisters. Turk J Pediatr; 2005 Apr-Jun;47(2):188-90
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  • [Title] Anaplastic large cell lymphoma in a child presenting with cutaneous nodules and blisters.
  • In another hospital, histopathological diagnosis of a skin biopsy was reported to be consistent with tuberculosis and she was treated with antimycobacterial drugs.
  • At our center, histopathological diagnosis was anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma (ALCL).
  • Eight months after initial admisision, she experienced cutaneous recurrence of disease while on maintenance protocol.
  • [MeSH-major] Lymphoma, Large B-Cell, Diffuse / diagnosis

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  • (PMID = 16052864.001).
  • [ISSN] 0041-4301
  • [Journal-full-title] The Turkish journal of pediatrics
  • [ISO-abbreviation] Turk. J. Pediatr.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Turkey
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79. Nguyen JT, Condron MR, Nguyen ND, De J, Medeiros LJ, Padula A: Anaplastic large cell lymphoma in leukemic phase: extraordinarily high white blood cell count. Pathol Int; 2009 May;59(5):345-53
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Anaplastic large cell lymphoma in leukemic phase: extraordinarily high white blood cell count.
  • Anaplastic large cell lymphoma (ALCL) is a distinct type of T/null-cell non-Hodgkin lymphoma that commonly involves nodal and extranodal sites.
  • The World Health Organization of lymphoid neoplasms recognizes two types: anaplastic lymphoma kinase (ALK) positive or ALK negative, the former as a result of abnormalities involving the ALK gene at chromosome 2p23.
  • Patients with ALCL rarely develop a leukemic phase of disease, either at the time of initial presentation or during the clinical course.
  • Described herein is a patient with ALK+ ALCL, small cell variant, associated with the t(2;5)(p23;q35), who initially presented with leukemic involvement and an extraordinarily high leukocyte count of 529 x 10(9)/L, which subsequently peaked at 587 x 10(9)/L.
  • Despite chemotherapy the patient died 2(1/2) months after diagnosis.
  • In the literature review 20 well-documented cases are identified of ALCL in leukemic phase reported previously, with a WBC ranging from 15 to 151 x 10(9)/L.
  • Leukemic phase of ALCL occurs almost exclusively in patients with ALK+ ALCL, most often associated with the small cell variant and the t(2;5)(p23;q35), similar to the present case.
  • Patients with leukemic phase ALK+ ALCL appear to have a poorer prognosis than most patients with ALK+ ALCL.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / blood. Lymphoma, Large-Cell, Anaplastic / pathology

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  • (PMID = 19432678.001).
  • [ISSN] 1440-1827
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Australia
  • [Chemical-registry-number] EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
  • [Number-of-references] 19
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80. Cho SG, Koh YB, Chang HS, Park G, Kang CS, Park JW, Min WS: Successful treatment with splenectomy and interferon alpha against recurred hemophagocytic syndrome in remission state of anaplastic large cell lymphoma following high-dose therapy and autologous peripheral blood stem cell transplantation. Eur J Haematol; 2005 Mar;74(3):259-62
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  • [Title] Successful treatment with splenectomy and interferon alpha against recurred hemophagocytic syndrome in remission state of anaplastic large cell lymphoma following high-dose therapy and autologous peripheral blood stem cell transplantation.
  • A 25-yr-old man had been diagnosed as having CD30(+) anaplastic large cell lymphoma associated with hemophagocytic syndrome (HS).
  • He received aggressive frontline chemotherapies and consolidation with autologous peripheral blood stem cell transplantation (PBSCT) following high-dose chemotherapy combined with splenic irradiation (720 cGy in fraction of 180 cGy).
  • However, HS recurred on day 50 of PBSCT without radiologic evidence of lymphoma relapse.
  • HS was completely resolved and he has been alive well and in complete remission (CR), 60 months after initial diagnosis.
  • [MeSH-major] Histiocytosis, Non-Langerhans-Cell / therapy. Interferon-alpha / therapeutic use. Lymphoma, Large-Cell, Anaplastic / complications. Splenectomy
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Disease-Free Survival. Humans. Male. Peripheral Blood Stem Cell Transplantation. Recurrence. Remission Induction / methods

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  • (PMID = 15693797.001).
  • [ISSN] 0902-4441
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Interferon-alpha
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81. Piccaluga PP, Gazzola A, Mannu C, Agostinelli C, Bacci F, Sabattini E, Sagramoso C, Piva R, Roncolato F, Inghirami G, Pileri SA: Pathobiology of anaplastic large cell lymphoma. Adv Hematol; 2010;:345053
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  • [Title] Pathobiology of anaplastic large cell lymphoma.
  • The authors revise the concept of anaplastic large cell lymphoma (ALCL) in the light of the recently updated WHO classification of Tumors of Hematopoietic and Lymphoid Tissues both on biological and clinical grounds.
  • The clinical features of ALCL are presented by underlining the difference in terms of response to therapy and survival between the ALK-positive and ALK-negative forms.

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  • (PMID = 21331150.001).
  • [ISSN] 1687-9112
  • [Journal-full-title] Advances in hematology
  • [ISO-abbreviation] Adv Hematol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3038421
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82. Bakshi NA, Ross CW, Finn WG, Valdez R, Ruiz R, Koujok K, Schnitzer B: ALK-positive anaplastic large cell lymphoma with primary bone involvement in children. Am J Clin Pathol; 2006 Jan;125(1):57-63
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  • [Title] ALK-positive anaplastic large cell lymphoma with primary bone involvement in children.
  • We describe the clinical, radiologic, and pathologic features of primary bone anaplastic large cell lymphoma (ALCL) in 3 boys.
  • Bone was the only site of disease in 2 cases; an associated partial lymph node was involved in case 3.
  • Differential diagnoses included osteomyelitis and small round cell tumors of childhood, particularly Ewing sarcoma.
  • Preoperatively, ALCL was not a diagnostic consideration in any case.
  • Two cases showed classic large pleomorphic cells; 1 showed a composite pattern with a distinct small cell component and the more typical large cell type.
  • Neoplastic cells in all cases showed strong CD30 and anaplastic lymphoma kinase expression with relatively weak epithelial membrane antigen positivity.
  • Two patients were disease-free at last follow-up (15 months and 11 years); 1 patient died of disseminated disease within a year of diagnosis.
  • ALCL should be considered a diagnostic possibility when evaluating neoplastic bone lesions in children.
  • Although expression of NSE in ALCL has not been emphasized in the literature, it is worth noting because it may pose a diagnostic pitfall.
  • [MeSH-major] Bone Neoplasms / pathology. Lymphoma, Large-Cell, Anaplastic / pathology. Protein-Tyrosine Kinases / analysis

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  • (PMID = 16482992.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 1.1.1.27 / L-Lactate Dehydrogenase; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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83. Escobosa Sánchez OM, Herrero Hernández A, Acha García T: [Endobronchial anaplastic large cell lymphoma in childhood]. An Pediatr (Barc); 2009 May;70(5):449-52
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  • [Title] [Endobronchial anaplastic large cell lymphoma in childhood].
  • [Transliterated title] Linfoma anaplásico de células grandes endobronquial en la infancia.
  • Anaplastic large cell lymphoma is a very rare disease in childhood.
  • The most common location of this lymphoma is lymph node and skin, with endobronchial involvement being extremely rare.
  • We report a case of a 10-year-old boy diagnosed by chance with an endobronchial anaplastic large cell lymphoma, while he was being investigated for a a benign bone disease, due to the initial absence of respiratory symptoms.
  • [MeSH-major] Bronchial Neoplasms. Lymphoma, Large-Cell, Anaplastic

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  • (PMID = 19375996.001).
  • [ISSN] 1695-4033
  • [Journal-full-title] Anales de pediatría (Barcelona, Spain : 2003)
  • [ISO-abbreviation] An Pediatr (Barc)
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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84. Cesaro S, Pillon M, Visintin G, Putti MC, Gazzola MV, D'Amore E, Scarzello G, Zanesco L, Messina C, Rosolen A: Unrelated bone marrow transplantation for high-risk anaplastic large cell lymphoma in pediatric patients: a single center case series. Eur J Haematol; 2005 Jul;75(1):22-6
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  • [Title] Unrelated bone marrow transplantation for high-risk anaplastic large cell lymphoma in pediatric patients: a single center case series.
  • OBJECTIVES: The use of allogeneic stem cell transplantation in NHL patients is not yet clearly defined, especially in children and adolescents, but this option offers the advantages of a tumor-free graft and the possible induction of a graft-vs.-tumor effect.
  • PATIENTS AND METHODS: We report the results of four consecutive pediatric patients affected by anaplastic large cell lymphoma (ALCL) and treated with allogeneic stem cell transplantation from an unrelated donor.
  • Graft-vs.-host disease (GVHD) prophylaxis consisted of cyclosporin, a short course of methotrexate and rabbit antithymocyte globulin.
  • This patient had been heavily pretreated during the course of the disease having suffered four relapses and had received a cumulative dose of bleomycin of 160 mg/m(2).
  • CONCLUSIONS: The increasing number of volunteer bone marrow donors and the reduced toxicity of unrelated stem cell transplantation, especially in children, make this therapeutic option worth more extensive investigation in the treatment of high-risk failure ALCL, although more data is needed to evaluate the long-term benefits.
  • In this regard, the presence of factors predictive of worst outcome such as an early relapse (within 12 months from diagnosis), a refractory or relapsing ALCL and the persistent detection on blood or bone marrow of nucleophosmin-anaplastic lymphoma kinase protein (NPM-ALK) transcript may help select the patients eligible to allogeneic related or unrelated stem cell transplantation.
  • [MeSH-major] Bone Marrow Transplantation. Lymphoma, Large B-Cell, Diffuse / therapy
  • [MeSH-minor] Adolescent. Adult. Antilymphocyte Serum / administration & dosage. Child. Child, Preschool. Cyclosporine / administration & dosage. Female. Follow-Up Studies. Graft vs Host Disease / prevention & control. Graft vs Leukemia Effect / drug effects. Graft vs Leukemia Effect / radiation effects. Histocompatibility Testing. Humans. Male. Risk Factors. Secondary Prevention. Transplantation Conditioning. Transplantation, Homologous. Whole-Body Irradiation


85. Chim CS, Ho J, Ooi GC, Choy C, Liang R: Primary anaplastic large cell lymphoma of the pancreas. Leuk Lymphoma; 2005 Mar;46(3):457-9
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  • [Title] Primary anaplastic large cell lymphoma of the pancreas.
  • We presented a patient with primary ALCL of the pancreas masquerading as a carcinoma of the head of pancreas.
  • She was scheduled for a Whipple's operation and the intra-operative frozen section showed anaplastic carcinoma.
  • The proper diagnosis was only derived later with CD30 immunohistochemical study.
  • This carries important implications on the management, as radical surgery is indicated in resectable carcinoma but not for a chemosensitive lymphoma.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / pathology. Pancreatic Neoplasms / pathology


86. de Jong D, Vasmel WL, de Boer JP, Verhave G, Barbé E, Casparie MK, van Leeuwen FE: Anaplastic large-cell lymphoma in women with breast implants. JAMA; 2008 Nov 5;300(17):2030-5
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  • [Title] Anaplastic large-cell lymphoma in women with breast implants.
  • CONTEXT: Recently, we identified 2 patients with anaplastic large T-cell lymphoma (ALCL) negative for tyrosine kinase anaplastic lymphoma kinase (ALK-negative) in the fibrous capsule of silicone breast prostheses, placed for cosmetic reasons.
  • Although an increased risk of ALCL in patients with breast prostheses has been speculated, no studies have been conducted so far.
  • OBJECTIVE: To determine whether ALCL risk is associated with breast prostheses.
  • DESIGN: A search for all patients with lymphoma in the breast diagnosed in The Netherlands between 1990 and 2006 was performed through the population-based nationwide pathology database.
  • Conditional logistic regression analysis was performed to estimate the relative risk of ALCL associated with breast prostheses.
  • SETTING AND PATIENTS: Eleven patients with breast ALCL were identified in the registry.
  • For each case patient with ALCL in the breast, we selected 1 to 5 controls with other lymphomas in the breast, matched on age and year of diagnosis.
  • MAIN OUTCOME MEASURE: Association between breast implants and ALCL of the breast.
  • RESULTS: The 11 patients with ALCL of the breast were diagnosed between 1994 and 2006 at a median age of 40 years (range, 24-68 years).
  • In 5 of these patients, bilateral silicone breast prostheses had been placed 1 to 23 years before diagnosis.
  • Lymphoma classes of 35 eligible control patients were 12 diffuse large B-cell lymphomas, including 1 T-cell rich B-cell lymphoma; 5 Burkitt lymphomas; 10 mucosa-associated lymphoid tissue-type lymphoma; 3 follicular lymphomas; 3 peripheral T-cell lymphomas; and 2 indolent B-cell lymphomas, unclassified.
  • One of 35 control patients had a breast implant placed before diagnosis of lymphoma.
  • The odds ratio for ALCL associated with breast prostheses was 18.2 (95% confidence interval, 2.1-156.8).
  • CONCLUSIONS: These preliminary findings suggest an association between silicone breast prostheses and ALCL, although the absolute risk is exceedingly low due to the rare occurrence of ALCL of the breast (11 cases in The Netherlands in 17 years).


87. Vaid R, Cohen B: Primary cutaneous CD30 positive anaplastic large cell lymphoma in an adolescent. Pediatr Dermatol; 2009 Nov-Dec;26(6):721-4
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  • [Title] Primary cutaneous CD30 positive anaplastic large cell lymphoma in an adolescent.
  • We present a case of a 14-year-old boy with a large ulcerated plaque on the scalp for 6 months, who was found to have primary cutaneous CD30-positive, anaplastic kinase-negative, anaplastic large cell lymphoma with post-auricular lymphadenopathy.
  • MRI, bone marrow biopsy, and laboratory data demonstrated no other systemic involvement.
  • Very few cases of primary cutaneous CD30-positive anaplastic large cell lymphoma in the pediatric population have been reported, and our case represents one of the first pediatric patients with local lymph node involvement.
  • [MeSH-major] Antigens, CD30 / metabolism. Lymphoma, Large B-Cell, Diffuse / pathology. Scalp / metabolism. Scalp / pathology. Skin Neoplasms / pathology

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  • (PMID = 20199449.001).
  • [ISSN] 1525-1470
  • [Journal-full-title] Pediatric dermatology
  • [ISO-abbreviation] Pediatr Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30; 0 / Antimetabolites, Antineoplastic; YL5FZ2Y5U1 / Methotrexate
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88. Riera L, Lasorsa E, Ambrogio C, Surrenti N, Voena C, Chiarle R: Involvement of Grb2 adaptor protein in nucleophosmin-anaplastic lymphoma kinase (NPM-ALK)-mediated signaling and anaplastic large cell lymphoma growth. J Biol Chem; 2010 Aug 20;285(34):26441-50
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Involvement of Grb2 adaptor protein in nucleophosmin-anaplastic lymphoma kinase (NPM-ALK)-mediated signaling and anaplastic large cell lymphoma growth.
  • Most anaplastic large cell lymphomas (ALCL) express oncogenic fusion proteins derived from chromosomal translocations or inversions of the anaplastic lymphoma kinase (ALK) gene.
  • Frequently ALCL carry the t(2;5) translocation, which fuses the ALK gene to the nucleophosmin (NPM1) gene.
  • The transforming activity mediated by NPM-ALK fusion induces different pathways that control proliferation and survival of lymphoma cells.
  • Grb2 is an adaptor protein thought to play an important role in ALK-mediated transformation, but its interaction with NPM-ALK, as well as its function in regulating ALCL signaling pathways and cell growth, has never been elucidated.
  • Furthermore, Grb2 did not bind to a single region but rather to different regions of NPM-ALK, mainly Tyr(152-156), Tyr(567), and a proline-rich region, Pro(415-417).
  • Finally, shRNA knockdown experiments showed that Grb2 regulates primarily the NPM-ALK-mediated phosphorylation of SHP2 and plays a key role in ALCL cell growth.
  • [MeSH-major] Cell Proliferation. GRB2 Adaptor Protein / metabolism. Lymphoma, Large-Cell, Anaplastic / pathology. Oncogene Proteins, Fusion / metabolism. Protein-Tyrosine Kinases / metabolism. Signal Transduction
  • [MeSH-minor] Binding Sites. Humans. Nuclear Proteins / genetics. Nuclear Proteins / metabolism. Phosphorylation. Protein Binding. Protein Tyrosine Phosphatase, Non-Receptor Type 11. Receptor Protein-Tyrosine Kinases. Translocation, Genetic. Tumor Cells, Cultured

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  • (PMID = 20554525.001).
  • [ISSN] 1083-351X
  • [Journal-full-title] The Journal of biological chemistry
  • [ISO-abbreviation] J. Biol. Chem.
  • [Language] eng
  • [Grant] International / European Research Council / / 242965
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / GRB2 Adaptor Protein; 0 / Nuclear Proteins; 0 / Oncogene Proteins, Fusion; 117896-08-9 / nucleophosmin; EC 2.7.1.- / p80(NPM-ALK) protein; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase; EC 3.1.3.48 / Protein Tyrosine Phosphatase, Non-Receptor Type 11
  • [Other-IDs] NLM/ PMC2924075
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89. Balwierz W, Czogała M, Czepko E: [Anaplastic large cell lymphoma associated with hemophagocytic lymphohistiocytosis: a case report and review of the literature]. Przegl Lek; 2010;67(6):436-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Anaplastic large cell lymphoma associated with hemophagocytic lymphohistiocytosis: a case report and review of the literature].
  • Here we present a case report of a 14 years old boy diagnosed with HLH before recognition of anaplastic large cell lymphoma (ALCL).
  • Finally, histopathological examination of a subsequently excised enlarged lymph node demonstrated lesions typical for ALCL.
  • First-line therapy, according to protocol for this type of lymphoma, was introduced.
  • He was qualified for allogeneic stem cell transplantation.
  • Occurrence of HLH can cause diagnostic difficulties, because it often masks the underlying disease, especially when it is associated with infection.
  • [MeSH-major] Lymphohistiocytosis, Hemophagocytic / diagnosis. Lymphohistiocytosis, Hemophagocytic / etiology. Lymphoma, Large-Cell, Anaplastic / complications. Lymphoma, Large-Cell, Anaplastic / diagnosis
  • [MeSH-minor] Adolescent. Adult. Child. Disease Progression. Fatal Outcome. Humans. Lymph Nodes / pathology. Male. Remission Induction. Stem Cell Transplantation


90. Koreen IV, Cho RI, Frueh BR, Elner VM: Primary cutaneous anaplastic large cell lymphoma of the medial canthus and orbit. Ophthal Plast Reconstr Surg; 2009 Jan-Feb;25(1):63-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary cutaneous anaplastic large cell lymphoma of the medial canthus and orbit.
  • Biopsy revealed CD30+ anaplastic large cell lymphoma.
  • Systemic evaluation for lymphoma was negative, and he underwent local radiotherapy.
  • The lesion regressed completely, and he has remained disease free for 7 months.
  • CD30+ anaplastic large cell lymphoma of the periocular skin and orbit are usually distinct, exceedingly rare entities; no reported cases had simultaneous involvement of both tissues.
  • The authors present the first reported case, to their knowledge, of simultaneous skin and orbital involvement by anaplastic large cell lymphoma.
  • [MeSH-major] Eyelid Neoplasms / pathology. Lymphoma, Large-Cell, Anaplastic / pathology. Orbital Neoplasms / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Antigens, CD3 / analysis. Antigens, CD30 / analysis. Humans. Immunoenzyme Techniques. Male. Middle Aged. Tomography, X-Ray Computed

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  • (PMID = 19273934.001).
  • [ISSN] 1537-2677
  • [Journal-full-title] Ophthalmic plastic and reconstructive surgery
  • [ISO-abbreviation] Ophthal Plast Reconstr Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD3; 0 / Antigens, CD30
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91. Sano F, Tasaka T, Nishimura H, Akiyama T, Kubo Y, Matsuhashi Y, Wada H, Sugihara T, Sadahira Y: Small cell variant of anaplastic large cell lymphoma diagnosed by a novel chromosomal abnormality t(2;5;3)(p23;q35;p21) of bone marrow cells. Pathol Int; 2008 Aug;58(8):494-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Small cell variant of anaplastic large cell lymphoma diagnosed by a novel chromosomal abnormality t(2;5;3)(p23;q35;p21) of bone marrow cells.
  • Because of the rarity and the morphological variations, small cell variant of anaplastic large cell lymphoma (ALCL) represents a diagnostic challenge.
  • Herein is reported a case of leukemic type of small cell variant of ALCL, in which the diagnosis was established by a cytogenetic analysis.
  • The initial differential diagnosis on bone marrow trephine biopsy sections included viral infection and peripheral T-cell lymphoma unspecified.
  • But a cytogenetic study on bone marrow cells indicated a novel complex translocation, t(2;5;3)(p23;q35;p21), which led to confirmation of anaplastic lymphoma kinase (ALK)-positive pleomorphic small to medium-sized cells scattered in bone marrow cells, on immunohistochemistry.
  • The patient achieved complete remission after four courses of combination chemotherapy, and received autologous peripheral stem cell transplantation (auto-PBSCT) after two additional courses of combination chemotherapy, but relapsed 2 months after auto-PBSCT in the bilateral lung.
  • Allogeneic stem cell transplantation led to a second remission.
  • This case demonstrates the diagnostic importance of cytogenetic study for malignant lymphoma involving bone marrow.
  • [MeSH-major] Bone Marrow Cells / pathology. Chromosomes, Human, 1-3 / genetics. Chromosomes, Human, Pair 5 / genetics. Lymphoma, Large-Cell, Anaplastic / diagnosis. Translocation, Genetic
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / metabolism. Chromosome Banding. Diagnosis, Differential. Female. Humans. Lymphoma, T-Cell, Peripheral / diagnosis. Protein-Tyrosine Kinases / immunology. Protein-Tyrosine Kinases / metabolism. Receptor Protein-Tyrosine Kinases. Remission Induction. Spectral Karyotyping. Virus Diseases / diagnosis

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  • (PMID = 18705769.001).
  • [ISSN] 1440-1827
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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92. Sevilla DW, Choi JK, Gong JZ: Mediastinal adenopathy, lung infiltrates, and hemophagocytosis: unusual manifestation of pediatric anaplastic large cell lymphoma: report of two cases. Am J Clin Pathol; 2007 Mar;127(3):458-64
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mediastinal adenopathy, lung infiltrates, and hemophagocytosis: unusual manifestation of pediatric anaplastic large cell lymphoma: report of two cases.
  • To date, only 1 report describes an anaplastic large cell lymphoma (ALCL) associated with hemophagocytosis in the pediatric population.
  • To better characterize this unusual manifestation of ALCL, we identified 2 additional cases.
  • Bone marrow biopsies and aspirates revealed striking hemophagocytosis but no ALCL.
  • Definitive diagnosis required lymph node biopsies that showed CD30+, anaplastic lymphoma kinase-l+ ALCL.
  • Both tumors responded to standard lymphoma chemotherapy.
  • These findings are similar to the first reported case and indicate that pediatric ALCL can manifest with an unusual constellation of symptoms consisting of hemophagocytosis, mediastinal or hilar adenopathy, and lung infiltrates.
  • [MeSH-major] Lung Diseases / pathology. Lymphatic Diseases / pathology. Lymphohistiocytosis, Hemophagocytic / pathology. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Activin Receptors, Type II / genetics. Antigens, CD30 / analysis. Antigens, CD8 / analysis. Child. Child, Preschool. Chromosomes, Human, Pair 2. Chromosomes, Human, Pair 5. Diagnosis, Differential. Fatal Outcome. Female. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Mediastinum. Translocation, Genetic

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  • (PMID = 17276937.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30; 0 / Antigens, CD8; EC 2.7.11.30 / ACVRL1 protein, human; EC 2.7.11.30 / Activin Receptors, Type II
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93. Bonvini P, Zorzi E, Mussolin L, Monaco G, Pigazzi M, Basso G, Rosolen A: The effect of the cyclin-dependent kinase inhibitor flavopiridol on anaplastic large cell lymphoma cells and relationship with NPM-ALK kinase expression and activity. Haematologica; 2009 Jul;94(7):944-55
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The effect of the cyclin-dependent kinase inhibitor flavopiridol on anaplastic large cell lymphoma cells and relationship with NPM-ALK kinase expression and activity.
  • BACKGROUND: The loss of cell cycle regulation due to abnormal function of cyclin-dependent kinases (cdk) occurs in tumors and leads to genetic instability of chemotherapy-resistant cells.
  • In this study, we investigated the effect of the cdk inhibitor flavopiridol in anaplastic large cell lymphomas, in which unrestrained proliferation depends on NPM-ALK tyrosine kinase activity.
  • DESIGN AND METHODS: Effects of flavopiridol were examined in ALK-positive and -negative anaplastic large cell lymphoma cells by means of immunoblotting and immunofluorescence analyses to assess cdk expression and activity, quantitative real time reverse transcriptase polymerase chain reaction to measure drug-induced changes in transcription, and FACS analyses to monitor changes in proliferation and survival.
  • RESULTS: Treatment with flavopiridol resulted in growth inhibition of anaplastic large cell lymphoma cells, along with accumulation of subG(1) cells and disappearance of S phase without cell cycle arrest.
  • This correlated with induction of cell death through rapid mitochondrial damage, inhibition of DNA synthesis, and down-regulation of anti-apoptotic proteins and transcripts.
  • Notably, flavopiridol was less active in ALK-positive cells, as apoptosis was observed at higher concentrations and later time points, and resistance to treatment was observed in cells maintaining NPM-ALK signaling.
  • NPM-ALK inhibition affected proliferation but not survival of anaplastic large cell lym-phoma cells, whereas it resulted in a dramatic increase in apoptosis when combined with flavopiridol.
  • CONCLUSIONS: This work provides the first demonstration that targeting cdk is effective against anaplastic large cell lymphoma cells, and proves the critical role of NPM-ALK in the regulation of responsiveness of tumor cells with cdk dysregulation.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Flavonoids / pharmacology. Gene Expression Regulation, Neoplastic. Lymphoma, Large-Cell, Anaplastic / drug therapy. Piperidines / pharmacology. Protein-Tyrosine Kinases / biosynthesis
  • [MeSH-minor] Apoptosis. Bromodeoxyuridine / pharmacology. Cell Cycle. Cell Separation. Cell Survival. Dose-Response Relationship, Drug. Humans. Reverse Transcriptase Polymerase Chain Reaction. Subcellular Fractions. Time Factors


94. Mühlhoff C, Rübben A, Gassler N, Megahed M: [Primary cutaneous CD30+ ALK(-) anaplastic large cell T-cell lymphoma]. Hautarzt; 2009 Dec;60(12):954-6
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  • [Title] [Primary cutaneous CD30+ ALK(-) anaplastic large cell T-cell lymphoma].
  • [Transliterated title] CD30(+) ALK(-) anaplastisch grosszelliges primär kutanes T-Zell-Lymphom.
  • Anaplastic large cell lymphomas (ALCL) are characterized by large, pleomorphic cells with a strong expression of cytokine receptor CD 30.
  • Based on clinical, histological and immunophenotypic criteria, the diagnosis of a primary cutaneous CD30(+) ALK(-) anaplastic large cell lymphoma was made.
  • [MeSH-major] Antigens, CD30 / analysis. Facial Neoplasms / pathology. Lymphoma, Primary Cutaneous Anaplastic Large Cell / pathology. Skin Neoplasms / pathology


95. Sebire NJ, Webb D, Ramsay AD: Anaplastic large cell lymphoma with ALK expression and presence of the t(2;5) translocation in a 5-month-old infant. Fetal Pediatr Pathol; 2005 Jan-Feb;24(1):63-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Anaplastic large cell lymphoma with ALK expression and presence of the t(2;5) translocation in a 5-month-old infant.
  • Anaplastic large cell lymphoma (ALCL) is a well-recognized subtype of non-Hodgkin lymphoma in childhood.
  • Several series report experience with the diagnosis and management of pediatric ALCL, the average age at diagnosis being 8 to 16 years, with a reported range of 1 to 15 years.
  • We present a case of ALCL affecting a 5-month-old infant in whom the diagnosis was confirmed by the nuclear and cytoplasmic immunohistochemical expression of ALK1, in addition to the presence of classical t(2;5)(p23;q35) translocation detected using reverse transcriptase-polymerase chain reaction.
  • This is the youngest case of ALCL thus far reported and hence expands the spectrum of infantile lymphoproliferative disorders.
  • [MeSH-major] Activin Receptors, Type I / biosynthesis. Lymphoma, Large B-Cell, Diffuse / genetics. Lymphoma, Large B-Cell, Diffuse / pathology. Translocation, Genetic
  • [MeSH-minor] Activin Receptors, Type II. Chromosomes, Human, Pair 2. Chromosomes, Human, Pair 5. Humans. Infant. Male

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  • (PMID = 16175752.001).
  • [ISSN] 1551-3815
  • [Journal-full-title] Fetal and pediatric pathology
  • [ISO-abbreviation] Fetal Pediatr Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.11.30 / ACVRL1 protein, human; EC 2.7.11.30 / Activin Receptors, Type I; EC 2.7.11.30 / Activin Receptors, Type II
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96. Sohatee MA: A case of anaplastic large cell lymphoma: when you hear hoof beats, sometimes consider zebras, not horses. BMJ Case Rep; 2009;2009

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of anaplastic large cell lymphoma: when you hear hoof beats, sometimes consider zebras, not horses.
  • These investigations revealed the diagnosis to be anaplastic large cell lymphoma.

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  • [Cites] Histopathology. 2002 Sep;41(3A):127-50 [12405944.001]
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  • (PMID = 21918660.001).
  • [ISSN] 1757-790X
  • [Journal-full-title] BMJ case reports
  • [ISO-abbreviation] BMJ Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC3029356
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97. Kodama K, Hokama M, Kawaguchi K, Tanaka Y, Hongo K: Primary ALK-1-negative anaplastic large cell lymphoma of the brain: case report and review of the literature. Neuropathology; 2009 Apr;29(2):166-71
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  • [Title] Primary ALK-1-negative anaplastic large cell lymphoma of the brain: case report and review of the literature.
  • Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma composed of CD30-positive cells.
  • Anaplastic lymphoma kinase (ALK) -1 positive ALCL frequently involves both lymph nodes and extranodal sites.
  • While primary extranodal involvement of ALK-1 negative ALCL is rare, this case is unique in that it is a case of primary ALK-1 negative ALCL of the brain.
  • The tumor was excised and histological diagnosis of primary ALK-1-negative ALCL was made.
  • Primary ALK-1-negative ALCL in this case showed aggressive clinical behavior and fatal outcome.
  • It is of great importance to avoid any delay in reaching an accurate diagnosis, as even primary ALCL of the brain is too seldom suspected clinically.
  • [MeSH-major] Activin Receptors, Type II / metabolism. Brain Neoplasms / metabolism. Brain Neoplasms / pathology. Lymphoma, Large-Cell, Anaplastic / metabolism. Lymphoma, Large-Cell, Anaplastic / pathology
  • [MeSH-minor] Adolescent. Aged. Blotting, Southern. Brain / metabolism. Brain / pathology. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Male. Middle Aged. Young Adult

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  • (PMID = 18564100.001).
  • [ISSN] 1440-1789
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Australia
  • [Chemical-registry-number] EC 2.7.11.30 / ACVRL1 protein, human; EC 2.7.11.30 / Activin Receptors, Type II
  • [Number-of-references] 29
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98. Marschalkó M, Eros N, Holló P, Hársing J, Bottlik G, Bátai A, Csukly Z, Masszi T, Szentirmai Z, Fodor J, Kárpáti S, Matolcsy A, Csomor J: Secondary ALK negative anaplastic large cell lymphoma in a patient with lymphomatoid papulosis of 40 years duration. Am J Dermatopathol; 2010 Oct;32(7):708-12
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  • [Title] Secondary ALK negative anaplastic large cell lymphoma in a patient with lymphomatoid papulosis of 40 years duration.
  • At age 44, regional lymph node manifestation of anaplastic lymphoma kinase (ALK) anaplastic large cell lymphoma (ALCL) developed.
  • Four years later, systemic relapse was detected which was refractory to therapy.
  • Histology and immunohistochemistry showed congruent characteristics of multiple skin and lymph node biopsies: diffuse mixed infiltrate with large, anaplastic CD30 cells.
  • Immunophenotype and microscopic morphology suggested a common origin of the different manifestations-however, this could not be proven due to lack of T-cell receptor (TCR) gamma gene rearrangement in most of the samples.
  • The diagnosis of ALK-negative systemic ALCL with cutaneous symptoms was set up at the second flare up, however, the possibility of primary cutaneous ALCL was not excluded steadily.
  • Lymphomatoid papulosis, primary cutaneous ALCL, and systemic ALK ALCL are 3 different entities but the separation of them cannot be solved without distinctive diagnostic tools.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / pathology. Lymphomatoid Papulosis / pathology. Neoplasms, Second Primary / pathology. Skin Neoplasms / pathology

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  • (PMID = 20644462.001).
  • [ISSN] 1533-0311
  • [Journal-full-title] The American Journal of dermatopathology
  • [ISO-abbreviation] Am J Dermatopathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.10.1 / anaplastic lymphoma kinase
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99. Newlove T, Loyd A, Patel R, Jelinek J, Latkowski JA: Primary cutaneous anaplastic large-cell lymphoma. Dermatol Online J; 2010;16(11):2
MedlinePlus Health Information. consumer health - Skin Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary cutaneous anaplastic large-cell lymphoma.
  • Primary cutaneous anaplastic large-cell lymphoma (ALCL) is a form of cutaneous T-cell lymphoma that is characterized by solitary or localized nodules or plaques.
  • Histopathologic features include a diffuse, non-epidermotropic infiltrate with cohesive sheets of large anaplastic CD30+ tumor cells.
  • This entity must be distinguished from systemic ALCL with cutaneous involvement and lymphomatoid papulosis.
  • Treatment modalities include clinical monitoring, radiation therapy, and surgical excision, with systemic chemotherapy reserved for disseminated or extracutaneous disease.
  • [MeSH-major] Lymphoma, Primary Cutaneous Anaplastic Large Cell / diagnosis. Skin Neoplasms / diagnosis
  • [MeSH-minor] Aged, 80 and over. Antigens, CD30 / metabolism. Combined Modality Therapy. Humans. Male. Treatment Outcome

  • Genetic Alliance. consumer health - Anaplastic Large Cell Lymphoma.
  • Genetic Alliance. consumer health - Lymphoma, large-cell.
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  • (PMID = 21163153.001).
  • [ISSN] 1087-2108
  • [Journal-full-title] Dermatology online journal
  • [ISO-abbreviation] Dermatol. Online J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30
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100. Müller J, Csóka M, Jakab Z, Ponyi A, Erlaky H, Kovács G: [Hungarian experience with non-Hodgkin's lymphoma in childhood]. Magy Onkol; 2006;50(3):253-9

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Hungarian experience with non-Hodgkin's lymphoma in childhood].
  • [Transliterated title] Gyermekkori non-Hodgkin-lymphoma kezelésével szerzett magyarországi tapasztalatok.
  • Between 1990 and 2004, 230 children with non-Hodgkin's lymphoma (NHL) were treated according to the Berlin-Frankfurt-Münster (BFM) protocols (NHL-BFM-90 and -95) in Hungary.
  • The male-to-female ratio was 2.59:1, the mean age at the time of diagnosis was 10 years and 1 month.
  • Ninety-one children had lymphoblastic/T-NHL (LB/T-NHL), 108 B-NHL and 31 anaplastic large cell lymphoma (ALCL).
  • Twenty-eight patients had relapse after a mean time of 13 months from the time of the initial diagnosis.
  • In the above mentioned period, 16 children underwent autologous stem-cell transplantation.
  • The 5-year OS and EFS rates were not statistically different in the three histology groups (OS: 71.6%+/-5%, 82.7%+/-4% and 80.3%+/-7%; EFS: 68.7%+/-5%, 81.1%+/-4% and 73.9%+/-8% in LB/T-NHL, B-NHL and ALCL, respectively).
  • We can conclude that non-Hodgkin's lymphoma has a quite good prognosis among the malignant pediatric diseases.
  • In case of relapse or residual disease, therapeutic results can be improved with stem-cell transplantation with or without immunotherapy.
  • [MeSH-major] Lymphoma, Non-Hodgkin
  • [MeSH-minor] Adolescent. Antineoplastic Agents / administration & dosage. Antineoplastic Agents / adverse effects. Child. Child, Preschool. Disease-Free Survival. Female. Humans. Hungary / epidemiology. Immunotherapy / methods. Incidence. Male. Prevalence. Recurrence. Remission Induction. Sex Distribution. Survival Rate. Treatment Outcome

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  • (PMID = 17099787.001).
  • [ISSN] 0025-0244
  • [Journal-full-title] Magyar onkologia
  • [ISO-abbreviation] Magy Onkol
  • [Language] hun
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Hungary
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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