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1. 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.
  • The optimal treatment strategy is unknown.
  • 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).
  • Total therapy was 48 weeks.
  • RESULTS: Eighty-six children (male 56%, female 44%) with non-localized ALCL (CD30+) were treated.
  • 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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2. Seidemann K, Tiemann M, Schrappe M, Yakisan E, Simonitsch I, Janka-Schaub G, Dörffel W, Zimmermann M, Mann G, Gadner H, Parwaresch R, Riehm H, Reiter A: Short-pulse B-non-Hodgkin lymphoma-type chemotherapy is efficacious treatment for pediatric anaplastic large cell lymphoma: a report of the Berlin-Frankfurt-Münster Group Trial NHL-BFM 90. Blood; 2001 Jun 15;97(12):3699-706
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  • [Title] Short-pulse B-non-Hodgkin lymphoma-type chemotherapy is efficacious treatment for pediatric anaplastic large cell lymphoma: a report of the Berlin-Frankfurt-Münster Group Trial NHL-BFM 90.
  • Anaplastic large-cell lymphoma (ALCL) accounts for approximately 10% of pediatric non-Hodgkin lymphoma (NHL).
  • Previous experience from NHL-Berlin-Frankfurt-Münster (BFM) trials indicated that the short-pulse B-NHL-type treatment strategy may also be efficacious for ALCL.
  • The purpose of this study was to test the efficacy of this protocol for treatment of childhood ALCL in a large prospective multicenter trial and to define risk factors.
  • Immunophenotype was T-cell in 40 patients, B-cell in 5, null in 31, and not determined in 13.
  • Extranodal manifestations were as follows: mediastinum, n = 28; lung, n = 13; skin, n = 16; soft tissue, n = 13; bone, n = 14; central nervous system, n = 1; bone marrow, n = 5.
  • After a cytoreductive prephase, treatment was stratified into 3 branches: patients in K1 (stage I and II resected) received three 5-day courses (methotrexate [MTX] 0.5 g/m(2), dexamethasone, oxazaphorins, etoposide, cytarabine, doxorubicin, and intrathecal therapy); patients in K2 (stage II nonresected and stage III) received 6 courses; patients in K3 (stage IV or multifocal bone disease) received 6 intensified courses including MTX 5 g/m(2), high-dose cytarabine/etoposide.
  • Events were as follows: progression during therapy, n = 2; progression or relapse after therapy, n = 20; second malignancy, n = 1.
  • It was concluded that short-pulse chemotherapy, stratified according to stage, is effective treatment for pediatric ALCL.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Lymphoma, Large-Cell, Anaplastic / drug therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Disease-Free Survival. Female. Germany. Humans. Immunophenotyping. Infant. Lymphoma, B-Cell / drug therapy. Male. Prospective Studies. Protein-Tyrosine Kinases / metabolism. Receptor Protein-Tyrosine Kinases. Recurrence. Risk Factors. Treatment Failure


3. Rosolen A, Pillon M, Garaventa A, Burnelli R, d'Amore ES, Giuliano M, Comis M, Cesaro S, Tettoni K, Moleti ML, Tamaro P, Visintin G, Zanesco L: Anaplastic large cell lymphoma treated with a leukemia-like therapy: report of the Italian Association of Pediatric Hematology and Oncology (AIEOP) LNH-92 protocol. Cancer; 2005 Nov 15;104(10):2133-40
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  • [Title] Anaplastic large cell lymphoma treated with a leukemia-like therapy: report of the Italian Association of Pediatric Hematology and Oncology (AIEOP) LNH-92 protocol.
  • BACKGROUND: Childhood anaplastic large cell lymphoma (ALCL) is a well defined entity with a rather poor prognosis.
  • Different approaches have been adopted in the treatment of ALCL in various cooperative trials, including short high-dose intensive therapy and leukemia-like protocols.
  • In the early 1990s, the Italian Association of Pediatric Hematology and Oncology (AIEOP) initiated a multicenter trial for the treatment of ALCL based on a modified LSA2-L2 protocol.
  • Treatment was comprised of an induction of remission phase, followed by consolidation and maintenance for a total duration of 24 months, independently of disease stage.
  • Therapy was well tolerated and hematologic toxicity was the most frequent toxicity.
  • CONCLUSIONS: The leukemia-like protocol AIEOP LNH-92 was found to be an effective treatment for childhood ALCL.
  • Its long duration may be beneficial to specific patient subgroups, but optimal treatment duration in ALCL remains to be elucidated.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Lymphoma, Large B-Cell, Diffuse / drug therapy. Lymphoma, Large B-Cell, Diffuse / mortality
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Cyclophosphamide / therapeutic use. Daunorubicin / therapeutic use. Disease-Free Survival. Female. Humans. Immunohistochemistry. Leukemia / therapy. Male. Methotrexate / therapeutic use. Neoplasm Staging. Prednisone / therapeutic use. Survival Analysis. Treatment Outcome. Vincristine / therapeutic use

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  • [Copyright] Copyright 2005 American Cancer Society
  • (PMID = 16211546.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide; VB0R961HZT / Prednisone; YL5FZ2Y5U1 / Methotrexate; ZS7284E0ZP / Daunorubicin; LSA2-L2 protocol
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4. Massimino M, Spreafico F, Luksch R, Giardini R: Prognostic significance of p80 and visceral involvement in childhood CD30 anaplastic large cell lymphoma (ALCL). Med Pediatr Oncol; 2001 Aug;37(2):97-102
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  • [Title] Prognostic significance of p80 and visceral involvement in childhood CD30 anaplastic large cell lymphoma (ALCL).
  • BACKGROUND: Between 1976 and 1998, CD30+anaplastic large cell lymphoma (ALCL) was diagnosed in 44 children (28 males, 16 females, age range 2.7-16.1 years, median 10).
  • Thereafter, patients presenting with visceral (lung, spleen, liver, gastro-intestinal tract) or mediastinal involvement were assigned to a high-risk treatment protocol with induction intensification.
  • The generation of these two risk-groups was the result of a retrospective analysis of clinical risk factors for therapy failure as previously reported [Massimino M, Gasparini M, Giardini R, Ann Oncol 1995;6:915-920].
  • PROCEDURE: We tested 15/21 cases of group A, and 18/22 of group B for p80 immunoreactivity in order to investigate a possible correlation between ALCL locations and NPM-ALK expression.
  • CONCLUSIONS: It is impossible to conclude anything about p80 positivity based on a series of 33/44 patients with childhood ALCL, neither about over-all prognosis nor about the role of visceral involvement.
  • [MeSH-major] Antigens, CD30 / immunology. Lymphoma, Large B-Cell, Diffuse / drug therapy. Neoplasm Metastasis. Protein-Tyrosine Kinases / biosynthesis
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Cohort Studies. Disease-Free Survival. Female. Follow-Up Studies. Gene Expression Regulation, Neoplastic. Humans. Immunoassay. Infant. Male. Prognosis. Retrospective Studies. Risk Factors. Treatment Outcome. Viscera / pathology

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  • [Copyright] Copyright 2001 Wiley-Liss, Inc.
  • (PMID = 11496346.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [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
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5. 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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  • [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.
  • Definitive diagnosis required lymph node biopsies that showed CD30+, anaplastic lymphoma kinase-l+ ALCL.
  • Both tumors responded to standard lymphoma chemotherapy.
  • One patient achieved complete remission, whereas the other patient died of complications after 2 cycles of therapy.
  • 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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6. 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
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  • [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).
  • 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.
  • [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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7. Makhonova LA, Peterson IS, Tupitsyn NN, Slugin AI, Matveeva II, Kiselev AV: [Diagnosis and treatment of large-cell anaplastic lymphoma in children]. Vestn Ross Akad Med Nauk; 2000;(6):8-10
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  • [Title] [Diagnosis and treatment of large-cell anaplastic lymphoma in children].
  • Thirty eight children with large-cell anaplastic lymphomas have been recently treated at the Pediatric Department of Malignant Lymphomas, Russian Cancer Research Center, Russian Academy of Medical Sciences.
  • Cytoimmunological studies have revealed that 24 patients had 4 types of cellular elements of large-cell anaplastic lymphoma: Kil(+), Kil(+)B, B-cell, and T-cell lymphomas in 11, 2, 8, and 3 children, respectively.
  • Intensive chemotherapy was performed in relation to the immunophenotype.

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  • (PMID = 10943152.001).
  • [ISSN] 0869-6047
  • [Journal-full-title] Vestnik Rossiiskoi akademii meditsinskikh nauk
  • [ISO-abbreviation] Vestn. Akad. Med. Nauk SSSR
  • [Language] RUS
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
  • [Chemical-registry-number] 0 / Antibodies, Neoplasm; 0 / Antigens, CD30
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8. Garner R, Li Y, Gray B, Zori R, Braylan R, Wall J, Hunger SP: Long-term disease control of refractory anaplastic large cell lymphoma with vinblastine. J Pediatr Hematol Oncol; 2009 Feb;31(2):145-7
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  • [Title] Long-term disease control of refractory anaplastic large cell lymphoma with vinblastine.
  • Anaplastic large cell lymphoma (ALCL) is a unique clinical and pathologic subtype of lymphoma characterized by the proliferation of large, highly pleomorphic CD30-positive cells.
  • Overall 70% to 80% of children with ALCL are cured with modern chemotherapy regimens, but the disease is often resistant to multiple therapies after relapse.
  • Single agent vinblastine therapy has been effective in some cases of refractory ALCL.
  • After relapse, the disease was refractory to multiagent chemotherapy, but has showed remarkable response to, and dependence on, single agent vinblastine treatment for almost 7 years.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / drug therapy. Vinblastine / therapeutic use
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Female. Humans. Recurrence. Remission Induction / methods. Salvage Therapy

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  • (PMID = 19194204.001).
  • [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
  • [Publication-country] United States
  • [Chemical-registry-number] 5V9KLZ54CY / Vinblastine
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9. Parker JR, López-Terrada D, Gresik MV, Vogel H, Baumgartner JE, Finegold MJ: Neutrophil-rich anaplastic large cell lymphoma of the skull presenting after head trauma. Pediatr Dev Pathol; 2001 Jul-Aug;4(4):397-401
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  • [Title] Neutrophil-rich anaplastic large cell lymphoma of the skull presenting after head trauma.
  • The presentation of anaplastic large cell lymphoma in bone is uncommon.
  • We report a case of anaplastic large cell lymphoma of the skull that was diagnosed after head trauma.
  • Histopathologically, the initial evaluation suggested osteomyelitis because of a mixed inflammatory infiltrate with large numbers of neutrophils.
  • The tumor cells had large, pleomorphic nuclei; these cells stained positively with antibodies to Ki-1 (CD 30), ALK-1, and EMA.
  • Fluorescence in situ hybridization (FISH) showed rearrangement of the ALK gene, which usually results from the t(2;5) translocation, present in most anaplastic large cell lymphomas.
  • The patient has tolerated chemotherapy and is free of disease 12 months later.
  • [MeSH-major] Antigens, CD30. Craniocerebral Trauma. Lymphoma, Large-Cell, Anaplastic / pathology. Neutrophils / pathology. Skull Neoplasms / pathology
  • [MeSH-minor] Activin Receptors. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cell Nucleus / chemistry. Cell Nucleus / genetics. Cell Nucleus / pathology. Child. DNA, Neoplasm / analysis. E2F6 Transcription Factor. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. Protein-Serine-Threonine Kinases / analysis. Protein-Serine-Threonine Kinases / genetics. Repressor Proteins / analysis. Transcription Factors / analysis

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  • (PMID = 11441342.001).
  • [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 / Antigens, CD30; 0 / DNA, Neoplasm; 0 / E2F6 Transcription Factor; 0 / E2F6 protein, human; 0 / Repressor Proteins; 0 / Transcription Factors; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 2.7.11.30 / Activin Receptors
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10. Perna AG, Jones DM, Duvic M: Lymphomatoid papulosis from childhood with anaplastic large-cell lymphoma of the small bowel. Clin Lymphoma; 2004 Dec;5(3):190-3
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  • [Title] Lymphomatoid papulosis from childhood with anaplastic large-cell lymphoma of the small bowel.
  • Lymphomatoid papulosis (LyP) is a lymphoproliferative disorder that exists on a spectrum of diseases with cutaneous CD30+ anaplastic large-cell lymphoma (ALCL).
  • Multiple treatment options are available, although none are curative.
  • A 43-year-old man developed lymphomatoid papulosis lesions at 3 years of age, which persisted into adulthood, and he later developed ALCL of the duodenum.
  • Treatment with standard CHOP (cyclophosphamide/doxorubicin/vincristine/prednisolone) chemotherapy resulted in complete remission of his gastrointestinal lymphoma and temporary improvement of his skin lesions.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Diphtheria Toxin / therapeutic use. Interleukin-2 / therapeutic use. Intestinal Neoplasms / pathology. Lymphoma, Large B-Cell, Diffuse / pathology. Lymphomatoid Papulosis / pathology. Recombinant Fusion Proteins / therapeutic use
  • [MeSH-minor] Adult. Child. Cyclophosphamide / administration & dosage. Doxorubicin / administration & dosage. Humans. Male. Prednisone / administration & dosage. Treatment Outcome. Vincristine / administration & dosage


11. 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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12. Ng A, Hobson R, Williams D, Morland B: Anaplastic large cell lymphoma of bone--is it a bad tumor? Pediatr Blood Cancer; 2007 Apr;48(4):473-6
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  • [Title] Anaplastic large cell lymphoma of bone--is it a bad tumor?
  • A teenage boy presented with a CD30-positive anaplastic large cell lymphoma (ALCL) affecting his scapula and was successfully treated with chemotherapy.
  • A further review of 11 ALCL cases with bony involvement treated in the UK since 1990, including two with primary bone disease, did not suggest an unfavorable treatment outcome.
  • [MeSH-major] Bone Neoplasms / pathology. Lymphoma, Large-Cell, Anaplastic / pathology. Scapula / pathology
  • [MeSH-minor] Activin Receptors, Type II / analysis. Activin Receptors, Type II / genetics. Adolescent. Antigens, CD30 / analysis. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / analysis. Biomarkers, Tumor / genetics. Child. Child, Preschool. Cohort Studies. Cyclophosphamide / administration & dosage. Cytarabine / administration & dosage. Dexamethasone / administration & dosage. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Female. Great Britain / epidemiology. Humans. Ifosfamide / administration & dosage. Infant. Magnetic Resonance Imaging. Male. Methotrexate / administration & dosage. Prognosis. Remission Induction. Treatment Outcome

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  • (PMID = 16078220.001).
  • [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 / Antigens, CD30; 0 / Biomarkers, Tumor; 04079A1RDZ / Cytarabine; 6PLQ3CP4P3 / Etoposide; 7S5I7G3JQL / Dexamethasone; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; EC 2.7.11.30 / ACVRL1 protein, human; EC 2.7.11.30 / Activin Receptors, Type II; UM20QQM95Y / Ifosfamide; YL5FZ2Y5U1 / Methotrexate
  • [Number-of-references] 11
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13. Palumbo G, Grana CM, Cocca F, De Santis R, Del Principe D, Baio SM, Mei R, Paganelli G: Pretargeted antibody-guided radioimmunotherapy in a child affected by resistant anaplastic large cell lymphoma. Eur J Haematol; 2007 Sep;79(3):258-62
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  • [Title] Pretargeted antibody-guided radioimmunotherapy in a child affected by resistant anaplastic large cell lymphoma.
  • Anaplastic large cell lymphoma (ALCL) is characterized by preferential paracortical and intrasinusoidal lymph node involvement by large anaplastic tumor cells expressing the CD30 antigen.
  • Up to 80% of pediatric patients with ALCL can be cured with multi-agent chemotherapeutic regimens.
  • Patients resistant to chemotherapy or suffering from early relapse have a poor prognosis and a poor chance of survival.
  • In these cases, the highly aggressive clinical course of ALCL, associated with systemic symptoms and extranodal involvement, has been treated with different approaches in various cooperative trials, including conventional chemotherapy and human stem cell transplantation (HSCT).
  • However, the optimal treatment has not yet been defined, in particular in cases of relapse.
  • More recently, radioimmunotherapy has been studied with encouraging results in cancer patients, including non-Hodgkin's lymphoma.
  • Here we describe the case of a pediatric ALCL, relapsing after HSCT, treated with pretargeted antibody-guided radioimmunotherapy, obtaining a complete remission, with excellent quality of life over the past 10 months.
  • [MeSH-major] Antibodies, Neoplasm / therapeutic use. Lymphoma, Large-Cell, Anaplastic / radionuclide imaging. Radioimmunotherapy / methods
  • [MeSH-minor] Child. Female. Hematopoietic Stem Cell Transplantation. Humans. Lung Neoplasms / secondary. Salvage Therapy / methods

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  • (PMID = 17655697.001).
  • [ISSN] 0902-4441
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antibodies, Neoplasm
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14. Ben Barak A, Elhasid R, Ben Itzhak O, Ben Arieh Y, Zaidman I, Haimi M, Bar-Joseph G, Ben Arush MW: Infant anaplastic lymphoma: case report and review of the literature. Pediatr Hematol Oncol; 2007 Jul-Aug;24(5):379-85

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Infant anaplastic lymphoma: case report and review of the literature.
  • Anaplastic large cell lymphoma (ALCL) is a well-known entity, but there are no data on prognosis according to the age of the patient, especially in infants.
  • Cervical lymph node biopsy revealed anaplastic lymphoma with positive staining to ALK 1 and TIA 1.
  • Immunophenotypic analysis of peripheral and bone marrow lymphoid cells showed an aberrant T-cell immunophenotype, including expression of CD3, CD45R0+, CD43+, and CD30+.
  • After leucophoresis, the child received chemotherapy according to the ALCL-99-EICNHL protocol, and was started on corticosteroids and cyclophosphamide, which resulted in marked improvement.
  • After the second course, WBC decreased to 6000/microL without tumor lysis syndrome, but the child developed bacterial and fungal disseminated infections and died of septic shock with multiorgan failure.
  • This report is of a rare case of infant anaplastic lymphoma and excellent response to treatment.
  • More reports of similar cases may determine the cause and prognosis of such children, helping to tailor therapy according to the age of the child and other prognostic factors, especially bone marrow involvement.
  • [MeSH-major] Lymphoma, Large-Cell, Anaplastic / diagnosis. Lymphoma, Large-Cell, Anaplastic / drug therapy

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  • (PMID = 17613884.001).
  • [ISSN] 1521-0669
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 17
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15. Lagmay J, Termuhlen A, Fung B, Ranalli M: Primary testicular presentation of ALK-1-negative anaplastic large cell lymphoma in a pediatric patient. J Pediatr Hematol Oncol; 2009 May;31(5):330-2
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  • [Title] Primary testicular presentation of ALK-1-negative anaplastic large cell lymphoma in a pediatric patient.
  • Anaplastic large cell lymphoma is a heterogeneous group of malignant non-Hodgkin lymphomas that occurs in up to 15% of all pediatric non-Hodgkin lymphomas.
  • It is characterized by B-symptoms and involvement of extranodal sites such as skin, bone, and soft tissue.
  • This brief report describes first reported case of pediatric primary testicular anaplastic large cell lymphoma in a 14-year-old boy.
  • After chemotherapy and unilateral radical orchiectomy, patient continues in complete remission.
  • [MeSH-major] Activin Receptors, Type II / metabolism. Lymphoma, Large B-Cell, Diffuse / metabolism. Lymphoma, Large B-Cell, Diffuse / pathology. Testicular Neoplasms / metabolism. Testicular Neoplasms / pathology
  • [MeSH-minor] Adolescent. Antigens, CD30 / metabolism. Biomarkers, Tumor / metabolism. Biopsy. Cell Nucleus / metabolism. Cell Nucleus / pathology. Golgi Apparatus / metabolism. Golgi Apparatus / pathology. Humans. Male. Orchiectomy. Testis / pathology. Testis / surgery

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  • (PMID = 19415011.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30; 0 / Biomarkers, Tumor; EC 2.7.11.30 / ACVRL1 protein, human; EC 2.7.11.30 / Activin Receptors, Type II
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16. Brugières L, Quartier P, Le Deley MC, Pacquement H, Perel Y, Bergeron C, Schmitt C, Landmann J, Patte C, Terrier-Lacombe MJ, Delsol G, Hartmann O: Relapses of childhood anaplastic large-cell lymphoma: treatment results in a series of 41 children--a report from the French Society of Pediatric Oncology. Ann Oncol; 2000 Jan;11(1):53-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Relapses of childhood anaplastic large-cell lymphoma: treatment results in a series of 41 children--a report from the French Society of Pediatric Oncology.
  • PURPOSE: To study response to chemotherapy and the outcome of children treated for a relapsed anaplastic large-cell lymphoma (ALCL) and to evaluate the role of bone marrow transplantation (BMT) in these patients.
  • First-line treatment consisted of intensive chemotherapy according to the COPAD protocol for the first series of 12 patients treated between 1975 and 1989 and to the SFOP (French Society of Pediatric Oncology) HM protocols for the 30 patients treated between 1989 and 1997.
  • Prolonged conventional chemotherapy based on vinblastine might, in some cases, be as efficient as short intensive treatment with ABMT.

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  • (PMID = 10690387.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 04079A1RDZ / Cytarabine; 11056-06-7 / Bleomycin; 5J49Q6B70F / Vincristine; 5V9KLZ54CY / Vinblastine; 7BRF0Z81KG / Lomustine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; EC 3.5.1.1 / Asparaginase; VB0R961HZT / Prednisone; YL5FZ2Y5U1 / Methotrexate
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17. Mora J, Filippa DA, Thaler HT, Polyak T, Cranor ML, Wollner N: Large cell non-Hodgkin lymphoma of childhood: Analysis of 78 consecutive patients enrolled in 2 consecutive protocols at the Memorial Sloan-Kettering Cancer Center. Cancer; 2000 Jan 1;88(1):186-97
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  • [Title] Large cell non-Hodgkin lymphoma of childhood: Analysis of 78 consecutive patients enrolled in 2 consecutive protocols at the Memorial Sloan-Kettering Cancer Center.
  • BACKGROUND: The authors report a study of pediatric patients with advanced diffuse large cell lymphoma (DLCL) who were treated with 2 consecutive regimens, LSA2-L2 and LSA4, over a 25-year-period at the Memorial Sloan-Kettering Cancer Center.
  • They also describe a comparative analysis of two subgroups retrospectively identified as having CD30 positive (+) anaplastic large cell lymphoma (ALCL) and CD30 negative (-) DLCL.
  • To the authors' knowledge, this study represents the longest follow-up on the largest series of uniformly treated pediatric DLCL patients reported to date.
  • Immunophenotypic data were obtained retrospectively for 52 patients using a panel of monoclonal antibodies against CD30, CD15, CD45, CD45Ro, CD43, epithelial membrane antigen, CD5, BCL-2, cyclin-D, and p53.
  • The recurrence rate was significantly higher in the CD30+ ALCL subgroup (33%) than in the CD30- DLCL group (0.04%).
  • Of 52 patients for whom immunophenotypic data were available, 28 had disease of B-cell lineage, 24 had disease of T-cell/null phenotype, 19 were CD30+ (36.
  • 5%), 18 had disease of T-cell phenotype, and 1 had disease of B-cell lineage.
  • CONCLUSIONS: The CD30- DLCL cases mostly were of B-cell lineage, had a small risk of treatment failure, and did not develop a recurrence off therapy.
  • A distinct clinical pattern was identified for the CD30+ ALCL group; although these tumors were of T-cell lineage and had a significantly higher rate of late recurrences (median follow-up of 24 months) they all were salvageable.
  • Based on the findings of the current study, the authors propose that T-cell CD30+ ALCL be addressed in the future according to equal dose intensity regimens in induction therapy, as is done for B-cell lymphomas; prolonged periods of maintenance chemotherapy, as is done for T-cell lymphoblastic lymphomas; and no central nervous system prophylaxis beyond the induction period unless other recognized risk factors are present.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Lymphoma, Large B-Cell, Diffuse / drug therapy
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor. Child. Child, Preschool. Disease-Free Survival. Drug Administration Schedule. Female. Humans. Incidence. Infant. Male. Neoplasm Staging. Neoplasms, Second Primary / complications. Retrospective Studies. Treatment Outcome


18. Chang BH, Stork L, Fan G: A unique case of adolescent CD56-negative extranodal NK/T-cell lymphoma, nasal type. Pediatr Dev Pathol; 2008 Jan-Feb;11(1):50-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A unique case of adolescent CD56-negative extranodal NK/T-cell lymphoma, nasal type.
  • Mature T-cell neoplasms are unusual in the pediatric population.
  • The majority of these neoplasms in the United States are anaplastic large cell lymphomas (ALCL) characterized by CD30 and anaplastic lymphoma kinase-1 expression.
  • Extranodal natural killer/T (NK/T)-cell lymphomas, nasal type, are extremely rare.
  • Extranodal NK/T-cell lymphomas often express CD56, are associated with Epstein-Barr virus, and are negative for CD30.
  • Clinically, extranodal NK/T-cell lymphomas are much more aggressive than ALCL, and require different treatment strategies.
  • The authors present an adolescent male with a CD56 negative extranodal NK/T-cell lymphoma, nasal type.
  • The lymphoma was partially positive for CD30, diffusely positive for EBV by in situ hybridization, and clonal for T-cell receptor gene rearrangement and cytogenetic abnormalities.
  • The patient was aggressively treated with chemotherapy, surgery, and radiation.
  • More than 2 years from completion of the therapy, the patient remains disease free.
  • This case highlights the importance and difficulty of accurate identification of this type of rare tumor.
  • We further present the literature review and discuss the diagnostic criteria for extranodal NK/T lymphoma using morphologic, immunologic, molecular, and cytogenetic information.
  • [MeSH-major] Antigens, CD56 / metabolism. Lymphoma, Extranodal NK-T-Cell / diagnosis. Lymphoma, Large-Cell, Anaplastic / diagnosis
  • [MeSH-minor] Adolescent. Antigens, CD30 / metabolism. Chromosome Aberrations. Diagnosis, Differential. Gene Rearrangement. Herpesvirus 4, Human / isolation & purification. Humans. In Situ Hybridization. Male. Nose / pathology. Receptors, Antigen, T-Cell / genetics

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  • [CommentIn] Pediatr Dev Pathol. 2008 Jul-Aug;11(4):325; author reply 326 [18839448.001]
  • (PMID = 18237230.001).
  • [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; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30; 0 / Antigens, CD56; 0 / Receptors, Antigen, T-Cell
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19. Williams DM, Hobson R, Imeson J, Gerrard M, McCarthy K, Pinkerton CR, United Kingdom Children's Cancer Study Group: Anaplastic large cell lymphoma in childhood: analysis of 72 patients treated on The United Kingdom Children's Cancer Study Group chemotherapy regimens. Br J Haematol; 2002 Jun;117(4):812-20
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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 childhood: analysis of 72 patients treated on The United Kingdom Children's Cancer Study Group chemotherapy regimens.
  • From June 1990 to June 1998, 72 patients with anaplastic large cell lymphoma (ALCL) were treated with short intensive multi-agent regimens [non-Hodgkin's lymphoma (NHL) 9000 and 9602].
  • Treatment for stage I disease consisted of eight courses (2 x vincristine, doxorubicin, prednisolone; 2 x methotrexate; 2 x cytarabine, thioguanine; and 2 x methotrexate etoposide).
  • For CNS-positive disease, treatment was intensified and contained methotrexate 8 g/m(2) and cytarabine 3 g/m(2).
  • Thirteen of these relapsed, with a median time to relapse from the start of treatment of 5 months (range 3-14).
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Lymphoma, Large-Cell, Anaplastic / drug therapy

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  • (PMID = 12060115.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 04079A1RDZ / Cytarabine; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; 9PHQ9Y1OLM / Prednisolone; FTK8U1GZNX / Thioguanine; YL5FZ2Y5U1 / Methotrexate
  • [Number-of-references] 26
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20. 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.
  • One patient later developed a restrictive pneumonopathy.
  • 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


21. Mori T, Takimoto T, Katano N, Kikuchi A, Tabuchi K, Kobayashi R, Ayukawa H, Kumagai MA, Horibe K, Tsurusawa M: Recurrent childhood anaplastic large cell lymphoma: a retrospective analysis of registered cases in Japan. Br J Haematol; 2006 Mar;132(5):594-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Recurrent childhood anaplastic large cell lymphoma: a retrospective analysis of registered cases in Japan.
  • This report presents a retrospective study of 26 Japanese children with recurrent anaplastic large cell lymphoma.
  • The patients who received allogeneic haematopoietic stem cell transplantation during second CR showed a superior outcome to other patients.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Lymphoma, Large-Cell, Anaplastic / drug therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Disease Progression. Female. Follow-Up Studies. Hematopoietic Stem Cell Transplantation. Humans. Infant. Japan. Male. Neoplasm Recurrence, Local / mortality. Remission Induction. Retrospective Studies. Survival Rate. Transplantation, Homologous. Treatment Outcome

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  • (PMID = 16445832.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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22. Vermeer MH, Bekkenk MW, Willemze R: Should primary cutaneous Ki-1(CD30)-positive anaplastic large cell lymphoma in childhood be treated with multiple-agent chemotherapy? J Am Acad Dermatol; 2001 Oct;45(4):638-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Should primary cutaneous Ki-1(CD30)-positive anaplastic large cell lymphoma in childhood be treated with multiple-agent chemotherapy?
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Lymphoma, Large-Cell, Anaplastic / drug therapy. Skin Neoplasms / drug therapy
  • [MeSH-minor] Adult. Antigens, CD30 / analysis. Child. Disease Progression. Humans. Male. Prognosis. Recurrence. Remission, Spontaneous. Skin Ulcer / etiology. Skin Ulcer / pathology

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  • [CommentOn] J Am Acad Dermatol. 1999 May;40(5 Pt 2):857-61 [10321635.001]
  • (PMID = 11568767.001).
  • [ISSN] 0190-9622
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Comment; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD30
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