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1. Bonkobara M, Saito T, Yamashita M, Tamura K, Yagihara H, Isotani M, Sato T, Washizu T: Blastic natural killer cell leukaemia in a dog--a case report. Vet J; 2007 Nov;174(3):659-62
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  • [Title] Blastic natural killer cell leukaemia in a dog--a case report.
  • A case of canine non-T, non-B lymphoid leukaemia was determined to be of natural killer (NK) cell lineage by detecting specific expression of canine CD56 mRNA by reverse transcriptase polymerase chain reaction analysis.
  • Although NK cells are usually considered to be morphologically large granular lymphocytes, the malignant NK cells in this case were agranular and blast-like, resembling human blastic NK cell leukaemia.
  • The prognosis of human NK cell leukaemia is usually poor.
  • [MeSH-major] Dog Diseases / diagnosis. Leukemia, Large Granular Lymphocytic / veterinary

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  • (PMID = 17113799.001).
  • [ISSN] 1090-0233
  • [Journal-full-title] Veterinary journal (London, England : 1997)
  • [ISO-abbreviation] Vet. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD56; 0 / Antineoplastic Agents; 0 / RNA, Messenger; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin
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2. Moubayed P, Leithäuser F, Binder T, Uppenkamp M, Feller AC: Two cases of primary malignant NK/T-cell lymphoma in the small intestine following an aggressive clinical course: Morphological, immunohistochemical, and molecular analysis. Leuk Lymphoma; 2007 Jul;48(7):1451-5
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  • [Title] Two cases of primary malignant NK/T-cell lymphoma in the small intestine following an aggressive clinical course: Morphological, immunohistochemical, and molecular analysis.
  • [MeSH-major] Ileal Neoplasms / diagnosis. Killer Cells, Natural / pathology. Lymphoma, T-Cell / diagnosis
  • [MeSH-minor] Adult. Aged. Cell Shape. Humans. Immunohistochemistry. Immunophenotyping. Male

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  • (PMID = 17613782.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
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3. Castelli R, Molteni M, Gianelli U, Cro L, Grimoldi MG, Cortelezzi A: Aggressive natural killer cell leukaemia with a complex karyotype: a case report. Ann Hematol; 2006 Jan;85(1):66-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Aggressive natural killer cell leukaemia with a complex karyotype: a case report.
  • [MeSH-major] Epstein-Barr Virus Infections / pathology. Killer Cells, Natural / pathology. Leukemia / pathology. Neoplasms, Second Primary / pathology

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  • (PMID = 16184393.001).
  • [ISSN] 0939-5555
  • [Journal-full-title] Annals of hematology
  • [ISO-abbreviation] Ann. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents; 0 / Antigens, CD; 0 / Antineoplastic Agents, Phytogenic; 0 / Immunosuppressive Agents; 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide; X4W7ZR7023 / Methylprednisolone
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4. Makishima H: [Analysis of chemokine receptor expression in aggressive NK-cell leukemia and chronic NK-cell lymphocytosis]. Rinsho Ketsueki; 2008 Jan;49(1):3-9
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  • [Title] [Analysis of chemokine receptor expression in aggressive NK-cell leukemia and chronic NK-cell lymphocytosis].
  • [MeSH-major] Chemokines / genetics. Chemokines / physiology. Killer Cells, Natural. Leukemia, Lymphoid / genetics. Leukemia, Lymphoid / pathology. Lymphocytosis / genetics. Lymphocytosis / pathology. Receptors, Chemokine / genetics. Receptors, Chemokine / physiology

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  • (PMID = 18277590.001).
  • [ISSN] 0485-1439
  • [Journal-full-title] [Rinshō ketsueki] The Japanese journal of clinical hematology
  • [ISO-abbreviation] Rinsho Ketsueki
  • [Language] jpn
  • [Publication-type] Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Chemokines; 0 / Receptors, CCR5; 0 / Receptors, Chemokine; 0 / Receptors, Interleukin-8A
  • [Number-of-references] 20
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5. Oshimi K, Kawa K, Nakamura S, Suzuki R, Suzumiya J, Yamaguchi M, Kameoka J, Tagawa S, Imamura N, Ohshima K, Kojya S, Iwatsuki K, Tokura Y, Sato E, Sugimori H, NK-cell Tumor Study Group: NK-cell neoplasms in Japan. Hematology; 2005 Jun;10(3):237-45
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  • [Title] NK-cell neoplasms in Japan.
  • Neoplasms putatively originating from precursor and mature natural killer (NK) cells are rare, and their clinical features are unclear.
  • A nationwide survey was performed in Japan to clarify the clinical features of these neoplasms diagnosed between 1994 and 1998, and data for 237 patients who met the criteria for putative NK cell-lineage neoplasms were analyzed.
  • Among them, 11 had myeloid/NK-cell precursor acute leukemia, 15 blastic NK-cell lymphoma, 21 precursor NK-cell acute lymphoblastic leukemia, 22 aggressive NK-cell leukemia/lymphoma, 149 nasal-type NK-cell lymphoma (123 nasal and 26 extranasal) and 19 chronic NK lymphocytosis.
  • The median overall survival time of patients with aggressive NK-cell leukemia/lymphoma was 2 months, which for chronic NK lymphocytosis was more than 8 years, and that for the other types of NK-cell neoplasms was between 6 and 22 months.
  • Nasal NK-cell lymphoma and extranasal NK-cell lymphoma share the same histology.
  • The age of affliction was the same, but the sex was different with males predominantly having nasal NK-cell lymphoma and females extranasal NK-cell lymphoma.
  • Patients with extranasal NK-cell lymphoma had the tendency to exhibit a more advanced state of disease, with significantly higher International Prognostic Index and LDH levels, and significantly lower hemoglobin and platelet levels.
  • Precursor NK-cell acute lymphoblastic leukemia and blastic NK-cell lymphoma were arbitrarily defined by the presence or absence of 30% or more of blastic cells in the bone marrow or peripheral blood, but there were no significant differences for affected age, gender, involved sites or prognosis.
  • Aggressive NK-cell leukemia/lymphoma and extranasal NK-cell lymphoma were arbitrarily defined by the presence or absence of 30% or more of large granular lymphocytes in the bone marrow or peripheral blood and it is possible that aggressive NK-cell leukemia/lymphoma is a leukemic phase of extranasal NK-cell lymphoma.
  • The incidence of skin involvement, however, was significantly higher for extranasal NK-cell lymphoma, suggesting that the two diseases are different.
  • In nasal NK-cell lymphoma, Epstein-Barr virus in tumor cells was detected in all patients tested, suggesting its causative role.
  • [MeSH-major] Killer Cells, Natural. Leukemia, Myeloid, Acute / mortality. Lymphoma / mortality. Nasopharyngeal Neoplasms / mortality


6. Lesport E, Baudhuin J, LeMaoult J, Sousa S, Doliger C, Carosella ED, Favier B: Human melanoma cell secreting human leukocyte antigen-G5 inhibit natural killer cell cytotoxicity by impairing lytic granules polarization toward target cell. Hum Immunol; 2009 Dec;70(12):1000-5
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  • [Title] Human melanoma cell secreting human leukocyte antigen-G5 inhibit natural killer cell cytotoxicity by impairing lytic granules polarization toward target cell.
  • The inhibitory role of HLA-G1 on NK cell cytotoxicity is well characterized; however, that of the HLA-G5 isoform secreted by tumor is poorly understood.
  • Our results indicate that the HLA-G5 isoform secreted by M8 melanoma cells is able to protect them from natural killer leukemia cell line (NKL) cytotoxicity.
  • Analysis of NKL/M8-HLA-G5 conjugates by confocal microscopy demonstrates that the inhibition of NKL cytotoxic activity resulted from an impairment of NKL actin reorganization and perforin granules polarization toward M8-HLA-G5 target cell.
  • This study also indicates that HLA-G5 soluble isoform remains evenly distributed in the cytoplasm of M8-HLA-G5 conjugated to NKL cells, suggesting that HLA-G5 does not require to polarize toward effector cell to induce efficient inhibition.
  • These results highlight the inhibitory mechanisms mediated through HLA-G5 leading to tumor escape from NK cell cytotoxicity.
  • [MeSH-major] Cytoplasmic Granules / immunology. HLA Antigens / immunology. Histocompatibility Antigens Class I / immunology. Killer Cells, Natural / immunology. Melanoma / immunology. Skin Neoplasms / immunology. Tumor Escape
  • [MeSH-minor] Actins / immunology. Actins / metabolism. Cell Line, Tumor. Cytotoxicity, Immunologic / immunology. HLA-G Antigens. Humans. Perforin / metabolism. Transfection

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  • (PMID = 19654030.001).
  • [ISSN] 1879-1166
  • [Journal-full-title] Human immunology
  • [ISO-abbreviation] Hum. Immunol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / HLA Antigens; 0 / HLA-G Antigens; 0 / Histocompatibility Antigens Class I; 126465-35-8 / Perforin
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7. Kishimoto S, Muramatsu M, Gokoh M, Oka S, Waku K, Sugiura T: Endogenous cannabinoid receptor ligand induces the migration of human natural killer cells. J Biochem; 2005 Feb;137(2):217-23
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  • [Title] Endogenous cannabinoid receptor ligand induces the migration of human natural killer cells.
  • In this study, we first examined the effects of 2-arachidonoylglycerol on the motility of human natural killer cells.
  • We found that 2-arachidonoylglycerol induces the migration of KHYG-1 cells (a natural killer leukemia cell line) and human peripheral blood natural killer cells.
  • The migration of natural killer cells induced by 2-arachidonoylglycerol was abolished by treating the cells with SR144528, a CB2 receptor antagonist, suggesting that the CB2 receptor is involved in the 2-arachidonoylglycerol-induced migration.
  • It is conceivable that the endogenous ligand for the cannabinoid receptor, that is, 2-arachidonoylglycerol, affects natural killer cell functions such as migration, thereby contributing to the host-defense mechanism against infectious viruses and tumor cells.
  • [MeSH-major] Arachidonic Acids / physiology. Cell Movement / physiology. Glycerides / physiology. Killer Cells, Natural / immunology. Receptor, Cannabinoid, CB2 / physiology
  • [MeSH-minor] Bornanes / pharmacology. Cell Line. Endocannabinoids. Humans. Ligands. Pyrazoles / pharmacology

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  • (PMID = 15749836.001).
  • [ISSN] 0021-924X
  • [Journal-full-title] Journal of biochemistry
  • [ISO-abbreviation] J. Biochem.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Arachidonic Acids; 0 / Bornanes; 0 / Endocannabinoids; 0 / Glycerides; 0 / Ligands; 0 / Pyrazoles; 0 / Receptor, Cannabinoid, CB2; 0 / SR 144528; 53847-30-6 / 2-arachidonylglycerol
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8. Suzuki R, Suzumiya J, Nakamura S, Kagami Y, Kameoka JI, Sakai C, Mukai H, Takenaka K, Yoshino T, Tsuzuki T, Sugimori H, Kawa K, Kodera Y, Oshimi K, NK-cell Tumor Study Group: Hematopoietic stem cell transplantation for natural killer-cell lineage neoplasms. Bone Marrow Transplant; 2006 Feb;37(4):425-31
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  • [Title] Hematopoietic stem cell transplantation for natural killer-cell lineage neoplasms.
  • Neoplasms of natural killer (NK)-lineage are rare.
  • Their prognosis is generally poor except for cases of solitary nasal NK-cell lymphoma.
  • The NK-cell Tumor Study Group performed a survey in Japan on patients diagnosed between 1994 and 1998.
  • The underlying diseases were myeloid/NK cell precursor acute leukemia (n = 4), blastic NK-cell lymphoma (n = 11), aggressive NK-cell leukemia (n = 3), and nasal-type extranodal NK-cell lymphoma (n = 22).
  • These findings suggest that the HSCT is a promising treatment strategy for NK-cell lineage.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation. Killer Cells, Natural / pathology. Leukemia / therapy. Lymphoma / therapy


9. Kwong YL: Natural killer-cell malignancies: diagnosis and treatment. Leukemia; 2005 Dec;19(12):2186-94
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  • [Title] Natural killer-cell malignancies: diagnosis and treatment.
  • Natural killer (NK)-cell malignancies are uncommon diseases.
  • Previously known as polymorphic reticulosis or angiocentric T-cell lymphomas, they are classified by the World Health Organization as NK/T-cell lymphoma, nasal type and aggressive NK-cell leukemia.
  • Pathologically, NK-cell lymphomas show a polymorphic neoplastic infiltrate with an angioinvasive and angiodestructive pattern.
  • Lymphoma cells are characteristically CD2+, CD56+ and cytoplasmic CD3epsilon+.
  • T-cell receptor gene is germline, and clonal Epstein-Barr virus (EBV) infection is almost invariably.
  • Clinically, they can be divided into nasal, non-nasal, and aggressive lymphoma/leukemia subtypes.
  • Most nasal NK-cell lymphomas present with stage I/II disease, and frontline radiotherapy is the most important key to successful treatment.
  • Chemotherapy is indicated for advanced nasal NK-cell lymphoma, and the non-nasal and aggressive subtypes.
  • High-dose chemotherapy with hematopoietic stem cell transplantation may be beneficial to selected patients.
  • [MeSH-major] Killer Cells, Natural / pathology. Leukemia. Lymphoma

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  • (PMID = 16179910.001).
  • [ISSN] 0887-6924
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Number-of-references] 83
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10. Oshimi K: Progress in understanding and managing natural killer-cell malignancies. Br J Haematol; 2007 Nov;139(4):532-44
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Progress in understanding and managing natural killer-cell malignancies.
  • The World Health Organization classification of haematolymphoid tumours recognizes three categories of natural killer (NK)-cell neoplasms: blastic NK-cell lymphoma, aggressive NK-cell leukaemia, and extranodal NK/T-cell lymphoma, nasal-type.
  • Recent studies indicate that CD4+CD56+ blastic NK-cell lymphoma is of plasmacytoid dendritic cell origin, and true tumours of precursor NK-cell origin may be present mainly in the CD4-CD56+ subset.
  • Myeloid/NK-cell precursor acute leukaemia may also develop from precursor NK cells.
  • However, because the developmental pathway of normal NK cells is not well understood, tumours of precursor NK-cell origin are not clearly identified.
  • Among mature NK-cell tumours, extranodal NK/T-cell lymphoma is relatively common in Asia and Latin America.
  • Aggressive NK-cell leukaemia is rare and has a poor prognosis.
  • Because NK-cell neoplasms are rare and difficult to manage, rigorous studies are required for their understanding and management.
  • [MeSH-major] Killer Cells, Natural / pathology. Leukemia, Lymphoid / therapy
  • [MeSH-minor] Acute Disease. Cell Lineage. Cell Transformation, Neoplastic / pathology. Chronic Disease. Humans. Neoplasm Staging. Preleukemia / pathology. Prognosis

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  • (PMID = 17916099.001).
  • [ISSN] 1365-2141
  • [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
  • [Number-of-references] 132
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11. Sugimoto K, Oshimi K: [Treatment of chronic lymphocytic leukemia and NK-cell leukemia/lymphoma]. Nihon Rinsho; 2007 Jan 28;65 Suppl 1:569-74
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  • [Title] [Treatment of chronic lymphocytic leukemia and NK-cell leukemia/lymphoma].
  • [MeSH-major] Leukemia, Lymphocytic, Chronic, B-Cell / therapy. Leukemia, T-Cell / therapy. Lymphoma, T-Cell / therapy
  • [MeSH-minor] Humans. Killer Cells, Natural

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  • (PMID = 17476757.001).
  • [ISSN] 0047-1852
  • [Journal-full-title] Nihon rinsho. Japanese journal of clinical medicine
  • [ISO-abbreviation] Nippon Rinsho
  • [Language] jpn
  • [Publication-type] Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 18
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12. Ando M, Sugimoto K, Kitoh T, Sasaki M, Mukai K, Ando J, Egashira M, Schuster SM, Oshimi K: Selective apoptosis of natural killer-cell tumours by l-asparaginase. Br J Haematol; 2005 Sep;130(6):860-8
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  • [Title] Selective apoptosis of natural killer-cell tumours by l-asparaginase.
  • We examined the effectiveness of various anti-tumour agents to natural killer (NK)-cell tumour cell lines and samples, which are generally resistant to chemotherapy, using flow cytometric terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end-labelling (TUNEL) assay.
  • Although NK-YS and NK-92 were highly resistant to various anti-tumour agents, l-asparaginase induced apoptosis in these two NK-cell lines.
  • NK-cell leukaemia/lymphoma and acute lymphoblastic leukaemia (ALL) samples were selectively sensitive to l-asparaginase and to doxorubicin (DXR) respectively.
  • Samples of chronic NK lymphocytosis, an NK-cell disorder with an indolent clinical course, were resistant to both drugs.
  • Our study clearly separated two major categories of NK-cell disorders and ALL according to the sensitivity to DXR and l-asparaginase.
  • At least in nasal-type NK-cell lymphoma, there was a good correlation among asparagine synthetase expression, in vitro sensitivity and clinical response to l-asparaginase.
  • In aggressive NK-cell leukaemia, although asparagine synthetase expression was high at both mRNA and protein levels, l-asparaginase induced considerable apoptosis.
  • We confirmed rather specific anti-tumour activity of l-asparaginase against NK-cell tumours in vitro, which provides an experimental background to the clinical use of l-asparaginase for NK-cell tumours.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Apoptosis / drug effects. Asparaginase / pharmacology. Killer Cells, Natural. Leukemia, T-Cell / pathology. Lymphoma, T-Cell / pathology
  • [MeSH-minor] Aspartate-Ammonia Ligase / biosynthesis. Aspartate-Ammonia Ligase / genetics. Cell Line, Tumor. Doxorubicin / pharmacology. Drug Resistance, Neoplasm. Humans. In Situ Nick-End Labeling. Lymphocytosis / pathology. RNA, Messenger / genetics. RNA, Neoplasm / genetics

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  • (PMID = 16156856.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 80168379AG / Doxorubicin; EC 3.5.1.1 / Asparaginase; EC 6.3.1.1 / Aspartate-Ammonia Ligase
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13. Schellekens J, Tilanus MG, Rozemuller EH: The elucidation of KIR2DL4 gene polymorphism. Mol Immunol; 2008 Apr;45(7):1900-6
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  • The killer cell immunoglobulin-like receptors (KIRs) on NK cells recognize defined groups of HLA class I alleles.
  • By this mechanism the NK cells fulfil a significant role in the first line of defense against infectious agents and cancer.
  • For the treatment of leukaemia this NK cell allorecognition is of great importance.

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  • (PMID = 18082267.001).
  • [ISSN] 0161-5890
  • [Journal-full-title] Molecular immunology
  • [ISO-abbreviation] Mol. Immunol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / DNA, Complementary; 0 / KIR2DL4 protein, human; 0 / Receptors, KIR2DL4
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14. Suzuki R: Treatment of advanced extranodal NK/T cell lymphoma, nasal-type and aggressive NK-cell leukemia. Int J Hematol; 2010 Dec;92(5):697-701
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  • [Title] Treatment of advanced extranodal NK/T cell lymphoma, nasal-type and aggressive NK-cell leukemia.
  • Extranodal NK/T cell lymphoma, nasal type (ENKL) with advanced stage and aggressive NK-cell leukemia (ANKL) are highly aggressive neoplasms with a dismal clinical outcome.
  • This is a major reason for the refractoriness to conventional chemotherapeutic regimens for malignant lymphoma containing anthracycline.
  • Optimal treatment scheme using such effective agents for these unfavorable NK-cell tumors should further be explored.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Killer Cells, Natural / pathology. Leukemia, T-Cell / therapy. Lymphoma, Extranodal NK-T-Cell / drug therapy

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  • (PMID = 21116747.001).
  • [ISSN] 1865-3774
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 6PLQ3CP4P3 / Etoposide; 7S5I7G3JQL / Dexamethasone; EC 3.5.1.1 / Asparaginase; UM20QQM95Y / Ifosfamide; YL5FZ2Y5U1 / Methotrexate
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15. Suzumiya J, Ohshima K, Tamura K, Karube K, Uike N, Tobinai K, Gascoyne RD, Vose JM, Armitage JO, Weisenburger DD, International Peripheral T-Cell Lymphoma Project: The International Prognostic Index predicts outcome in aggressive adult T-cell leukemia/lymphoma: analysis of 126 patients from the International Peripheral T-Cell Lymphoma Project. Ann Oncol; 2009 Apr;20(4):715-21
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  • [Title] The International Prognostic Index predicts outcome in aggressive adult T-cell leukemia/lymphoma: analysis of 126 patients from the International Peripheral T-Cell Lymphoma Project.
  • BACKGROUND: The International Peripheral T-cell Lymphoma Project was organized to better understand the T-cell and natural killer (NK) cell lymphomas, and our task is to present the clinicopathologic correlations and therapeutic results for adult T-cell leukemia/lymphoma (ATL).
  • PATIENTS AND METHODS: Among 1153 patients with T-cell or NK cell lymphomas, 126 patients (9.6%) with ATL were represented in this project.
  • All were categorized as aggressive ATL, i.e. acute or lymphoma type, and 87% fell into the lymphoma type.
  • CONCLUSION: Patients with aggressive ATL have a poor clinical outcome and the IPI is a useful model for predicting outcome in ATL of the lymphoma type.

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  • (PMID = 19150954.001).
  • [ISSN] 1569-8041
  • [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
  • [Publication-country] England
  • [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; 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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16. Tong H, Ren Y, Liu H, Xiao F, Mai W, Meng H, Qian W, Huang J, Mao L, Tong Y, Wang L, Qian J, Jin J: Clinical characteristics of T-cell lymphoma associated with hemophagocytic syndrome: comparison of T-cell lymphoma with and without hemophagocytic syndrome. Leuk Lymphoma; 2008 Jan;49(1):81-7
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  • [Title] Clinical characteristics of T-cell lymphoma associated with hemophagocytic syndrome: comparison of T-cell lymphoma with and without hemophagocytic syndrome.
  • T-cell lymphoma-associated hemophagocytic syndrome (T-LAHS) has been frequently reported in Asian countries and is considered with extremely poor prognosis.
  • To summarize its clinical characteristics and explore its early diagnosis and treatment, we retrospectively analyzed the records of 113 patients with aggressive T cell lymphoma, of which 28 were associated with LAHS.
  • According to WHO classification (2001), 22 cases were classified into peripheral T-cell lymphoma (unspecified), 2 into extranodal NK/T-cell lymphoma, and 4 into systemic anaplastic large cell lymphoma.
  • Repeating biopsies of multiple parts of bone marrow may help diagnosis.
  • [MeSH-major] Lymphohistiocytosis, Hemophagocytic / pathology. Lymphoma, T-Cell / complications. Lymphoma, T-Cell / pathology

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  • (PMID = 18203016.001).
  • [ISSN] 1029-2403
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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17. Nava VE, Jaffe ES: The pathology of NK-cell lymphomas and leukemias. Adv Anat Pathol; 2005 Jan;12(1):27-34
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  • [Title] The pathology of NK-cell lymphomas and leukemias.
  • Natural killer (NK) cell lymphomas and leukemias are a rare but clinically important group of neoplasms.
  • Most of these tumors are aggressive, with a high rate of mortality.
  • They include extranodal NK/T-cell lymphomas of nasal type and aggressive NK-cell leukemias.
  • A closely related entity seen mainly in children is hydroa vacciniforme-like lymphoma, which also is EBV positive.
  • The differential diagnosis of NK-cell malignancies includes fulminant EBV-associated T-cell lymphoproliferative disorder, a condition referred to in the past as fatal infectious mononucleosis.
  • Benign proliferations of NK cells can be seen in association with viral infection.
  • The disease formerly referred to as blastic NK-cell lymphoma is now considered to be a malignancy derived from a dendritic cell precursor.
  • [MeSH-major] Epstein-Barr Virus Infections / pathology. Killer Cells, Natural / pathology. Leukemia / pathology. Leukemia / physiopathology. Lymphocyte Subsets / pathology. Lymphoma / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Child. Diagnosis, Differential. Herpesvirus 4, Human. Humans. Immunohistochemistry

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  • (PMID = 15614162.001).
  • [ISSN] 1072-4109
  • [Journal-full-title] Advances in anatomic pathology
  • [ISO-abbreviation] Adv Anat Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 89
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18. Zhang K, Prichard JW, Brown RE: Blastic natural killer (NK) cell leukemia (agranular CD4+CD56+ leukemia). Ann Clin Lab Sci; 2006;36(2):212-5
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  • [Title] Blastic natural killer (NK) cell leukemia (agranular CD4+CD56+ leukemia).
  • Blastic NK cell lymphoma is a rare hematolymphoid neoplasm.
  • This report illustrates an unusual presentation of this entity, namely as a primary leukemia, but without skin lesions.
  • [MeSH-major] Bone Marrow Neoplasms / diagnosis. Killer Cells, Natural. Leukemia, Lymphoid / diagnosis. Precursor Cell Lymphoblastic Leukemia-Lymphoma / diagnosis

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  • (PMID = 16682521.001).
  • [ISSN] 0091-7370
  • [Journal-full-title] Annals of clinical and laboratory science
  • [ISO-abbreviation] Ann. Clin. Lab. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD4; 0 / Antigens, CD56
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19. Sokol L, Loughran TP Jr: Large granular lymphocyte leukemia. Curr Hematol Malig Rep; 2007 Oct;2(4):278-82
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  • [Title] Large granular lymphocyte leukemia.
  • Clonal diseases of large granular lymphocytes (LGLs) represent a spectrum of clinically rare lymphoproliferative malignancies arising from either mature T-cell (CD3(+)) or natural killer (NK)-cell (CD3(-)) lineages.
  • The clinical behavior of these disorders ranges from indolent to very aggressive.
  • Patients with symptomatic indolent T-cell or NK-cell LGL leukemia are usually treated with immunosuppressive therapies; in contrast, aggressive T-cell or NK-cell LGL leukemias require intensive chemotherapy regimens.
  • [MeSH-major] Leukemia, Large Granular Lymphocytic
  • [MeSH-minor] Adolescent. Adult. Antibodies, Monoclonal / therapeutic use. Antibodies, Monoclonal, Humanized. Antibodies, Neoplasm / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Clinical Trials as Topic. Combined Modality Therapy. Disease Progression. Female. Humans. Immunophenotyping. Killer Cells, Natural / pathology. Leukemia, T-Cell / complications. Leukemia, T-Cell / epidemiology. Leukemia, T-Cell / immunology. Leukemia, T-Cell / pathology. Leukemia, T-Cell / therapy. Male. Middle Aged. Myelodysplastic Syndromes / pathology. Neutropenia / etiology. Opportunistic Infections / etiology. Stem Cell Transplantation

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  • (PMID = 20425381.001).
  • [ISSN] 1558-822X
  • [Journal-full-title] Current hematologic malignancy reports
  • [ISO-abbreviation] Curr Hematol Malig Rep
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 3A189DH42V / alemtuzumab
  • [Number-of-references] 39
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20. Kawamata N, Inagaki N, Mizumura S, Sugimoto KJ, Sakajiri S, Ohyanagi-Hara M, Oshimi K: Methylation status analysis of cell cycle regulatory genes (p16INK4A, p15INK4B, p21Waf1/Cip1, p27Kip1 and p73) in natural killer cell disorders. Eur J Haematol; 2005 May;74(5):424-9
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  • [Title] Methylation status analysis of cell cycle regulatory genes (p16INK4A, p15INK4B, p21Waf1/Cip1, p27Kip1 and p73) in natural killer cell disorders.
  • Natural killer (NK) cell disorders are rare diseases.
  • In this study we analyze the methylation status of the genes associated with cell cycle regulation, including p16INK4A, p15INK4B, p21/Waf1/Cip1, p27/Kip1, p73, and p14ARF, by methylation specific (MS) PCR and/or bisulfite sequencing.
  • We examined 29 cases of NK cell disorders (five aggressive NK cell leukemia/lymphoma, three blastic NK cell lymphoma/leukemia, five nasal NK cell lymphoma, three myeloid/NK cell precursor acute leukemia, 13 chronic NK lymphocytosis).
  • We found methylation of the first exon of the p16INK4A gene in two cases (one aggressive, one blastic), and methylation of the p14ARF gene in one aggressive NK cell leukemia.
  • MS-PCR suggested that the p73 and p21 genes were methylated in seven cases, respectively (p73: one blastic, one nasal, five chronic; p21: one myeloid/NK, one aggressive, one nasal, and four chronic); bisulfite sequencing confirmed that methylated alleles of these genes were dominant in the samples except three cases (one myeloid/NK, one aggressive, and one chronic) in which methylated alleles of the p21 genes were less than 34% of all alleles.
  • These results suggested that inactivation of the cell cycle regulatory genes by DNA methylation could be associated with tumorigenesis in NK cell disorders, not only aggressive subtypes but also chronic subtype.
  • [MeSH-major] Cell Cycle / genetics. Cell Cycle Proteins / genetics. Cyclin-Dependent Kinase Inhibitor p16 / genetics. DNA Methylation. DNA-Binding Proteins / genetics. Killer Cells, Natural / physiology. Leukemia / genetics. Lymphoma / genetics. Nuclear Proteins / genetics. Tumor Suppressor Proteins / genetics

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  • (PMID = 15813917.001).
  • [ISSN] 0902-4441
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / CDKN1A protein, human; 0 / CDKN2B protein, human; 0 / Cell Cycle Proteins; 0 / Cyclin-Dependent Kinase Inhibitor p15; 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / Cyclin-Dependent Kinase Inhibitor p21; 0 / DNA Primers; 0 / DNA-Binding Proteins; 0 / Nuclear Proteins; 0 / Tumor Suppressor Proteins; 0 / tumor suppressor protein p73
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21. Iqbal J, Kucuk C, Deleeuw RJ, Srivastava G, Tam W, Geng H, Klinkebiel D, Christman JK, Patel K, Cao K, Shen L, Dybkaer K, Tsui IF, Ali H, Shimizu N, Au WY, Lam WL, Chan WC: Genomic analyses reveal global functional alterations that promote tumor growth and novel tumor suppressor genes in natural killer-cell malignancies. Leukemia; 2009 Jun;23(6):1139-51
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  • [Title] Genomic analyses reveal global functional alterations that promote tumor growth and novel tumor suppressor genes in natural killer-cell malignancies.
  • Natural killer (NK)-cell malignancies are among the most aggressive lymphoid neoplasms with very poor prognosis.
  • We performed array comparative genomic hybridization analysis on a number of NK cell lines and primary tumors to gain better understanding of the pathogenesis and tumor biology of these malignancies.
  • We also obtained transcriptional profiles of genes residing in these regions and compared them with normal and activated NK cells.
  • Genes downregulated in regions of loss included many transcription factors or repressors, tumor suppressors or negative regulators of the cell cycle.
  • Reversal of methylation by Decitabine induced expression of PRDM1 and cell death.
  • [MeSH-major] Comparative Genomic Hybridization. Crystallins / genetics. Gene Expression Regulation, Neoplastic. Genes, Tumor Suppressor. Killer Cells, Natural / pathology. Lymphoma / genetics. Membrane Proteins / genetics. Microtubule-Associated Proteins / genetics. Repressor Proteins / genetics
  • [MeSH-minor] Adult. Aged. Cell Proliferation. Chromosomes, Human, Pair 6. Gene Expression Profiling. Humans. Middle Aged. Tumor Cells, Cultured

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  • (PMID = 19194464.001).
  • [ISSN] 1476-5551
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / 5 U01 CA114778; United States / NCRR NIH HHS / RR / P20 RR016469
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / AIM1 protein, human; 0 / ATG5 protein, human; 0 / Crystallins; 0 / Membrane Proteins; 0 / Microtubule-Associated Proteins; 0 / Repressor Proteins; 138415-26-6 / PRDM1 protein, human
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22. Matano S, Terahata S, Nakamura S, Kobayashi K, Sugimoto T: CD56-positive acute lymphoblastic leukemia. Acta Haematol; 2005;114(3):160-3
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  • [Title] CD56-positive acute lymphoblastic leukemia.
  • We report a patient with lung cancer who developed CD56-positive acute lymphoblastic leukemia.
  • There were no rearrangements of T-cell receptor (TCR)-beta, TCR-gamma, or immunoglobulin heavy chain.
  • Systemic examination did not detect any tumors other than pulmonary adenocarcinoma, and the patient was diagnosed as having acute natural killer (NK) cell leukemia.
  • The course of the disease was complicated by a lung abscess, and the patient died 3 months after the diagnosis.
  • We considered that the diagnosis was blastic NK cell lymphoma/leukemia subtype.
  • However, it actually was myeloid/NK cell precursor leukemia subtype that weakly expressed CD13.
  • [MeSH-major] Antigens, CD56 / metabolism. Precursor Cell Lymphoblastic Leukemia-Lymphoma / immunology
  • [MeSH-minor] Aged. Humans. Immunophenotyping. Killer Cells, Natural / classification. Killer Cells, Natural / immunology. Killer Cells, Natural / pathology. Male

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  • [Copyright] (c) 2005 S. Karger AG, Basel
  • (PMID = 16227680.001).
  • [ISSN] 0001-5792
  • [Journal-full-title] Acta haematologica
  • [ISO-abbreviation] Acta Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antigens, CD56
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23. Pullarkat VA, Medeiros LJ, Brynes RK: Body cavity-based presentation of natural killer cell lymphoma. Leuk Lymphoma; 2005 Feb;46(2):293-6
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  • [Title] Body cavity-based presentation of natural killer cell lymphoma.
  • We describe an unusual case of a 31-year-old Mexican woman who presented with pleural and peritoneal effusions involved by Epstein-Barr virus-positive non-Hodgkin's lymphoma of natural killer (NK)-cell lineage.
  • Thus, this case clinically mimicked body cavity-based lymphoma.
  • Extranodal NK/T-cell lymphoma of nasal type is the current designation for these neoplasms in the recently proposed World Health Organization classification of lymphoid neoplasms.
  • These tumors previously have been referred to many other names, including lethal midline granuloma, midline malignant reticulosis, polymorphic reticulosis, angiocentric immunoproliferative lesion, and angiocentric lymphoma.
  • Nasal-type NK/T-cell lymphomas typically involve the nasal region, but may involve other extranodal sites, such as skin and gastrointestinal tract.
  • The malignant cytologic features and the presence of azurophilic granules within the cell cytoplasm observed in Wright-Giemsa-stained cytocentrifuge preparations led to immunophenotypic and molecular genetic studies that were essential in establishing the correct diagnosis.
  • As demonstrated in the case reported, extranodal NK/T-cell lymphomas of nasal-type can be clinically aggressive and may be associated with paraneoplastic phenomena.
  • [MeSH-major] Killer Cells, Natural / pathology. Lymphoma, T-Cell / pathology. Nose Neoplasms / diagnosis. Pleural Effusion, Malignant / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Herpesvirus 4, Human / isolation & purification. Humans. Pleural Cavity / pathology

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  • (PMID = 15621817.001).
  • [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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24. Gogia A, Kakar A, Byotra SP, Bhargav M: Aggressive natural killer cell leukaemia: a rare and fatal disorder. J Assoc Physicians India; 2010 Nov;58:702-4
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  • [Title] Aggressive natural killer cell leukaemia: a rare and fatal disorder.
  • Natural killer (NK) cell neoplasms, which include extra-nodal NK/T-cell lymphoma (nasal and extra-nasal) and aggressive NK cell leukaemia, are generally rare, but they are more common in people of Oriental, Mexican and South American descent.
  • These neoplasms are highly aggressive, and show a strong association with Epstein-Barr virus.
  • Aggressive NK cell leukaemia affects younger patients, who present with poor general condition, fever, and disseminated disease; they often die within a short time from systemic disease or complications such as multi-organ failure.
  • Aggressive NK cell leukaemia must be distinguished from T-cell large granular lymphocyte leukaemia and indolent NK cell lympho-proliferative disorder, both of which are indolent.
  • We present a case of young Asian male with aggressive NK cell leukaemia who presented with a poor general condition and disseminated disease.
  • The patient had a rapidly progressive disease and died within weeks of diagnosis.
  • [MeSH-major] Killer Cells, Natural / pathology. Leukemia / pathology. Lymphoma / pathology

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  • (PMID = 21510468.001).
  • [ISSN] 0004-5772
  • [Journal-full-title] The Journal of the Association of Physicians of India
  • [ISO-abbreviation] J Assoc Physicians India
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Antibodies, Anti-Idiotypic; 0 / anti-IgG
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25. Brodtman DH, Rosenthal DW, Redner A, Lanzkowsky P, Bonagura VR: Immunodeficiency in children with acute lymphoblastic leukemia after completion of modern aggressive chemotherapeutic regimens. J Pediatr; 2005 May;146(5):654-61
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  • [Title] Immunodeficiency in children with acute lymphoblastic leukemia after completion of modern aggressive chemotherapeutic regimens.
  • OBJECTIVE: To determine the prevalence, duration, and a potential cause of humoral defect(s) in children with acute lymphoblastic leukemia (ALL) at least 1 year after completion of chemotherapy.
  • Children with non-protective titers to these microbial antigens were re-vaccinated and re-studied after anamnestic vaccine challenge.
  • Longitudinally, many titers fluctuate between protective and non-protective antibody responses after re-immunization.
  • T-, B-, and NK-cell numbers and proliferative responses to mitogens were all normal.
  • Pediatricians, oncologists, or both should periodically monitor humoral immunity after chemotherapy and re-vaccinate these children, as needed, to ensure prolonged immunoprotection.
  • [MeSH-major] Antibody Formation / drug effects. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Asparaginase / therapeutic use. Communicable Diseases / immunology. Daunorubicin / therapeutic use. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy. Prednisone / therapeutic use. Vincristine / therapeutic use

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  • (PMID = 15870670.001).
  • [ISSN] 0022-3476
  • [Journal-full-title] The Journal of pediatrics
  • [ISO-abbreviation] J. Pediatr.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; EC 3.5.1.1 / Asparaginase; VB0R961HZT / Prednisone; ZS7284E0ZP / Daunorubicin; PVDA protocol
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26. Wilentz SE: Blastic natural killer-cell lymphoma: a case report. Cutis; 2006 Jan;77(1):42-4
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  • [Title] Blastic natural killer-cell lymphoma: a case report.
  • This article presents a case of blastic natural killer (NK)-cell lymphoma.
  • Most cases, including the one presented here, follow an aggressive clinical course.
  • [MeSH-major] Killer Cells, Natural / pathology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology

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  • (PMID = 16475495.001).
  • [ISSN] 0011-4162
  • [Journal-full-title] Cutis
  • [ISO-abbreviation] Cutis
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor
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27. Ito T, Makishima H, Nakazawa H, Kobayashi H, Shimodaira S, Nakazawa Y, Kitano K, Matsuda K, Hidaka E, Ishida F: Promising approach for aggressive NK cell leukaemia with allogeneic haematopoietic cell transplantation. Eur J Haematol; 2008 Aug;81(2):107-11
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Promising approach for aggressive NK cell leukaemia with allogeneic haematopoietic cell transplantation.
  • OBJECTIVES: Aggressive natural killer cell leukaemia (ANKL) is a malignant disorder of mature NK cells with a poor prognosis, for which no effective therapeutic approach has been established.
  • We investigated the role of allogeneic haematopoietic cell transplantion (allo-HCT) in ANKL.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation / methods. Killer Cells, Natural. Leukemia, Lymphoid / therapy
  • [MeSH-minor] Adult. Bone Marrow Transplantation. Cord Blood Stem Cell Transplantation. Female. Herpesvirus 4, Human. Humans. Male. Polymerase Chain Reaction. Transplantation Conditioning / methods. Transplantation, Homologous. Treatment Outcome. Viral Load


28. Cabrera ME, Eizuru Y, Itoh T, Koriyama C, Tashiro Y, Ding S, Rey S, Akiba S, Corvalan A: Nasal natural killer/T-cell lymphoma and its association with type "i"/XhoI loss strain Epstein-Barr virus in Chile. J Clin Pathol; 2007 Jun;60(6):656-60
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  • [Title] Nasal natural killer/T-cell lymphoma and its association with type "i"/XhoI loss strain Epstein-Barr virus in Chile.
  • BACKGROUND: Nasal T/natural killer (NK)-cell lymphoma is an aggressive type of non-Hodking's lymphoma associated with Epstein-Barr virus (EBV) and striking geographical variations worldwide.
  • AIM: To characterise nasal NK/T-cell lymphoma associated with genotypes of EBV in Chile, a Latin American country, where multiple strains of EBV, including two new recombinant strains, in healthy individuals were recently found.
  • METHODS: Cases with diagnosis of primary nasal lymphoma were selected for histological and immunohistochemical analysis (CD3, CD3e, CD4, CD8, CD79a, CD56, CD57 and TIA-1) and in-situ hybridisation, serology and genotyping analysis for EBV.
  • RESULTS: Out of 22 cases, 9 (41%) cases fulfilled the World Health Organization criteria for nasal NK/T-cell lymphoma; of these 7 (78%) cases were positive for EBV.
  • CONCLUSION: Although nasal NK/T-cell lymphomas from Chile share similar clinicopathological features, high association with EBV and unfavourable prognosis with those described elsewhere, genotype analysis shows that the new recombinant type "i"/XhoI loss strain might contribute to explain the intermediate incidence of nasal NK/T-cell lymphomas in Latin America.
  • [MeSH-major] Epstein-Barr Virus Infections / complications. Herpesvirus 4, Human / genetics. Killer Cells, Natural / pathology. Lymphoma, T-Cell / virology. Nose Neoplasms / virology

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  • (PMID = 16775124.001).
  • [ISSN] 0021-9746
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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  • [Other-IDs] NLM/ PMC1955082
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29. Gross SA, Zhu X, Bao L, Ryder J, Le A, Chen Y, Wang XQ, Irons RD: A prospective study of 728 cases of non-Hodgkin lymphoma from a single laboratory in Shanghai, China. Int J Hematol; 2008 Sep;88(2):165-73
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A prospective study of 728 cases of non-Hodgkin lymphoma from a single laboratory in Shanghai, China.
  • Diagnosis and classification was established in a single laboratory according to the 2001 WHO classification system.
  • The frequency of non-Hodgkin lymphoma was 87.6% (n = 728) and Hodgkin lymphoma was 12.4% (n = 103).
  • The most prevalent NHL subtypes diagnosed using WHO criteria were diffuse large B cell lymphoma (DLBCL), precursor B lymphoblastic leukemia/lymphoma and chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL).
  • Although a low incidence has been reported in some Asian populations, CLL/SLL was commonly encountered, indicating that chronic lymphoid neoplasms are not rare in Shanghai.
  • Consistent with previous reports, our findings indicate a decrease in the frequency of follicular lymphoma and an increase in T cell neoplasms compared to the West.
  • Precursor T lymphoblastic leukemia/lymphoma, anaplastic large T cell lymphoma, aggressive NK cell leukemia, angioimmunoblastic T cell lymphoma and peripheral T cell lymphoma were prominent subtypes of T cell NHL.
  • [MeSH-major] Asian Continental Ancestry Group / statistics & numerical data. Lymphoma, Non-Hodgkin / classification. Lymphoma, Non-Hodgkin / ethnology

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  • (PMID = 18648906.001).
  • [ISSN] 0925-5710
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / DNA, Neoplasm
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30. Bang SM, Kim YK, Park YH, Sohn SK, Lee JJ, Cho EK, Ryoo BY, Chung IJ, Yoon SS, Kim HJ, Lee JH, Yoon HJ, Park S: High-dose therapy and autologous stem cell transplantation in Korean patients with aggressive T/NK-cell lymphoma. Leuk Lymphoma; 2005 Nov;46(11):1599-1604
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] High-dose therapy and autologous stem cell transplantation in Korean patients with aggressive T/NK-cell lymphoma.
  • The proportion of aggressive T/NK-cell lymphoma in Korea is larger than in the West, and it shows a lower response to conventional chemotherapy and poorer survival than diffuse large B-cell lymphoma.
  • This study was undertaken to evaluate the response rate and survival and to document the prognostic factors in patients with T/NK-cell lymphoma who have undergone high-dose therapy (HDT).
  • Eligibility for the study was a mature T/NK-cell lymphoma with initially poor risk (as high or high intermediate risk on age-adjusted International Prognostic Index) or relapsed cases.
  • Twelve patients had unspecified peripheral T-cell lymphomas, 7 anaplastic large-cell lymphomas, 6 nasal T/NK-cell lymphomas, and 3 angioimmunoblastic T-cell lymphomas.
  • Absolute neutrophil count ( > 500/microl) recovered at a median 11 days after autologous stem cell transplantation in 26 patients.
  • Therefore, an initial approach with effective induction and HDT may result in a better outcome in T/NK-cell lymphoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Hematopoietic Stem Cell Transplantation / methods. Killer Cells, Natural / pathology. Lymphoma, T-Cell / therapy

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  • (PMID = 16334486.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] England
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31. Jaccard A, Petit B, Girault S, Suarez F, Gressin R, Zini JM, Coiteux V, Larroche C, Devidas A, Thiéblemont C, Gaulard P, Marin B, Gachard N, Bordessoule D, Hermine O: L-asparaginase-based treatment of 15 western patients with extranodal NK/T-cell lymphoma and leukemia and a review of the literature. Ann Oncol; 2009 Jan;20(1):110-6
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  • [Title] L-asparaginase-based treatment of 15 western patients with extranodal NK/T-cell lymphoma and leukemia and a review of the literature.
  • BACKGROUND: Extranodal natural killer (NK)/T-cell lymphoma, nasal type, and aggressive NK-cell leukemia are highly aggressive diseases with a poor outcome.
  • CONCLUSION: These data, although retrospective, confirm the excellent activity of L-asparaginase-containing regimens in refractory extranodal NK/T-cell lymphoma and aggressive NK-cell leukemia.
  • First-line L-asparaginase combination therapy for extranodal NK/T-cell lymphoma and aggressive NK-cell leukemia should be tested in prospective trials.

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  • (PMID = 18701429.001).
  • [ISSN] 1569-8041
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Evaluation Studies; Journal Article; Multicenter Study; Review
  • [Publication-country] England
  • [Chemical-registry-number] EC 3.5.1.1 / Asparaginase
  • [Number-of-references] 26
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32. Ham MF, Ko YH: Natural killer cell neoplasm: biology and pathology. Int J Hematol; 2010 Dec;92(5):681-9
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  • [Title] Natural killer cell neoplasm: biology and pathology.
  • Natural killer (NK) cell neoplasm is a heterogeneous disease group.
  • In the latest World Health Organization (WHO) classification of tumours of hematopoietic and lymphoid tissues (2008), disease entities considered as NK-cell derivation include NK-lymphoblastic leukemia/lymphoma, chronic lymphoproliferative disorders of NK cells, aggressive NK-cell leukemia, and extranodal NK-cell lymphoma, nasal-type.
  • Despite recent advances in NK-cell research, which have expanded our understanding of the biology of NK-cell neoplasm, it cannot yet be sharply delineated from myeloid neoplasms and T-cell neoplasms even in some "well-known" entity, such as extranodal NK/T-cell lymphoma.
  • This review describes current knowledge of the biology of NK cells and pathology of NK neoplasms as classified in the 2008 WHO classification of tumours of hematopoietic and lymphoid tissues.
  • [MeSH-major] Killer Cells, Natural / pathology. Neoplasms / pathology
  • [MeSH-minor] Humans. Lymphoma, Extranodal NK-T-Cell / pathology. Lymphoproliferative Disorders / pathology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology

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  • (PMID = 21132576.001).
  • [ISSN] 1865-3774
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Japan
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33. Lozzi GP, Massone C, Citarella L, Kerl H, Cerroni L: Rimming of adipocytes by neoplastic lymphocytes: a histopathologic feature not restricted to subcutaneous T-cell lymphoma. Am J Dermatopathol; 2006 Feb;28(1):9-12
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  • [Title] Rimming of adipocytes by neoplastic lymphocytes: a histopathologic feature not restricted to subcutaneous T-cell lymphoma.
  • Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma of the skin presenting with histopathologic features simulating those of a lobular panniculitis.
  • The presence of neoplastic T-lymphocytes forming a rim around the individual fat cells in the subcutaneous lobules, so-called "rimming" of adipocytes, is considered a characteristic morphologic feature of this type of cutaneous lymphoma.
  • In this study we reviewed a series of 45 biopsy specimens of primary and secondary cutaneous B- and T-cell lymphomas and one of myeloid leukemia involving the subcutaneous tissues and showing rimming of adipocytes (subcutaneous panniculitis-like T-cell lymphoma: n = 16; mycosis fungoides, tumor stage: n = 3; aggressive epidermotropic CD8(+) T-cell lymphoma: n = 2; cutaneous gamma/delta T-cell lymphoma: n = 4; extranodal NK/T-cell lymphoma, nasal type: n = 4; cutaneous medium-large pleomorphic T-cell lymphoma, NOS: n = 5; CD4(+)/CD56(+) hematodermic neoplasm (blastic NK-cell lymphoma): n = 7; secondary cutaneous large B-cell lymphoma: n = 3; secondary cutaneous lymphoplasmacytic lymphoma: n = 1; specific cutaneous manifestations of acute myelogenous leukemia: n = 1).
  • We could demonstrate that rimming of adipocytes by neoplastic cells can be recognized not only in subcutaneous panniculitis-like T-cell lymphoma, but also in several different entities of malignant lymphoma with skin involvement.
  • Rimming of adipocytes should not be considered specific of subcutaneous panniculitis-like T-cell lymphoma.
  • [MeSH-major] Adipocytes / pathology. Lymphoma, B-Cell / pathology. Lymphoma, T-Cell / pathology. Skin Neoplasms / pathology. T-Lymphocytes / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / metabolism. Female. Gene Rearrangement. Genes, T-Cell Receptor gamma / genetics. Humans. Immunoglobulin Heavy Chains / genetics. Male. Middle Aged. Panniculitis / metabolism. Panniculitis / pathology. Receptors, Antigen, T-Cell, gamma-delta / genetics. Receptors, Antigen, T-Cell, gamma-delta / metabolism

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  • (PMID = 16456318.001).
  • [ISSN] 0193-1091
  • [Journal-full-title] The American Journal of dermatopathology
  • [ISO-abbreviation] Am J Dermatopathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Immunoglobulin Heavy Chains; 0 / Receptors, Antigen, T-Cell, gamma-delta
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34. Oka K, Nagayama R, Mori N: Epstein-Barr virus-associated proliferative disorder presenting as Hodgkin's lymphoma and developing as aggressive natural killer-cell leukemia 19 years later: a case report of composite lymphoma. Pathol Res Pract; 2009;205(10):730-4
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  • [Title] Epstein-Barr virus-associated proliferative disorder presenting as Hodgkin's lymphoma and developing as aggressive natural killer-cell leukemia 19 years later: a case report of composite lymphoma.
  • We describe a patient who was diagnosed as having classic Hodgkin's lymphoma at 29 years of age, and aggressive natural killer-cell leukemia at 48 years.
  • Hodgkin and Reed-Sternberg cells in the lymph node expressed CD30, CD15, T-cell intracellular antigen-1 (TIA-1), perforin, granzyme B, and Epstein-Barr virus-encoded RNA (EBER).
  • Natural killer-cell leukemia cells in the bone marrow expressed cytoplasmic CD3epsilon, TIA-1, perforin, granzyme B, and EBER, and some neoplastic cells expressed CD56 (123C3).
  • Fluorescence-activated cell sorter (FACS) analysis showed that neoplastic cells expressed CD56.
  • Neither a rearrangement band of the T-cell receptor gene nor that of the immunoglobulin heavy chain gene was detected.
  • [MeSH-major] Epstein-Barr Virus Infections / complications. Hodgkin Disease / pathology. Leukemia, Large Granular Lymphocytic / pathology. Neoplasms, Second Primary / pathology

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  • (PMID = 19269751.001).
  • [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
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35. Nakashima Y, Tagawa H, Suzuki R, Karnan S, Karube K, Ohshima K, Muta K, Nawata H, Morishima Y, Nakamura S, Seto M: Genome-wide array-based comparative genomic hybridization of natural killer cell lymphoma/leukemia: different genomic alteration patterns of aggressive NK-cell leukemia and extranodal Nk/T-cell lymphoma, nasal type. Genes Chromosomes Cancer; 2005 Nov;44(3):247-55
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  • [Title] Genome-wide array-based comparative genomic hybridization of natural killer cell lymphoma/leukemia: different genomic alteration patterns of aggressive NK-cell leukemia and extranodal Nk/T-cell lymphoma, nasal type.
  • Natural killer (NK) cell lymphomas/leukemias are highly aggressive lymphoid malignancies, but little is known about their genomic alterations, and thus there is an urgent need for identification and analysis of NK cell lymphomas/leukemias.
  • We performed an array CGH analysis for 27 NK-cell lymphoma/leukemia cases that were classified into two disease groups based on the World Health Organization Classification (10 aggressive NK-cell leukemia cases and 17 extranodal NK/T-cell [NK/T] lymphomas, nasal type).
  • The recurrent regions characteristic of the aggressive NK-cell leukemia group compared with those of the extranodal NK/T lymphoma, nasal-type group, were gain of 1q and loss of 7p15.1-p22.3 and 17p13.1.
  • Recurrent regions characteristic of the extranodal NK/T lymphoma, nasal-type group, compared with those of the other group were gain of 2q, and loss of 6q16.1-q27, 11q22.3-q23.3, 5p14.1-p14.3, 5q34-q35.3, 1p36.23-p36.33, 2p16.1-p16.3, 4q12, and 4q31.3-q32.1.
  • Our results can be expected to provide further insights into the genetic basis of lymphomagenesis and the clinicopathologic features of NK-cell lymphomas/leukemias.
  • [MeSH-major] Chromosome Aberrations. Chromosomes, Human / genetics. Genome, Human. Killer Cells, Natural / pathology. Leukemia, T-Cell / genetics. Lymphoma, T-Cell / genetics. Nose Neoplasms / genetics


36. Ryder J, Wang X, Bao L, Gross SA, Hua F, Irons RD: Aggressive natural killer cell leukemia: report of a Chinese series and review of the literature. Int J Hematol; 2007 Jan;85(1):18-25
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  • [Title] Aggressive natural killer cell leukemia: report of a Chinese series and review of the literature.
  • Aggressive natural killer cell leukemia (ANKL) is a rare Epstein-Barr virus (EBV)-associated fulminating disease that is widely disseminated at diagnosis.
  • Because of its typically extranodal presentation, differing degrees of NK cell involvement, and varying bone marrow pathology, ANKL can be confused with a reactive process.
  • All cases were positive for EBV early region protein and negative for latent membrane protein 1, and all had a germline T-cell receptor gene configuration.
  • Peripheral blood counts were variable, with severe thrombocytopenia being the most frequently encountered abnormality (7 of 9 cases).
  • Because of its aggressive course, rapid and accurate diagnosis of ANKL is essential for a better understanding of the etiology, pathogenesis, and treatment of the disease.
  • [MeSH-major] Killer Cells, Natural / virology. Leukemia, T-Cell / diagnosis. Leukemia, T-Cell / epidemiology
  • [MeSH-minor] Adult. Aged. Blood Cell Count. Bone Marrow Diseases / pathology. China. Cytogenetic Analysis. Female. Herpesvirus 4, Human. Humans. Immunophenotyping. Male. Middle Aged. Thrombocytopenia

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  • (PMID = 17261497.001).
  • [ISSN] 0925-5710
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
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37. Shen L, Au WY, Guo T, Wong KY, Wong ML, Tsuchiyama J, Yuen PW, Kwong YL, Liang RH, Srivastava G: Proteasome inhibitor bortezomib-induced apoptosis in natural killer (NK)-cell leukemia and lymphoma: an in vitro and in vivo preclinical evaluation. Blood; 2007 Jul 1;110(1):469-70
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  • [Title] Proteasome inhibitor bortezomib-induced apoptosis in natural killer (NK)-cell leukemia and lymphoma: an in vitro and in vivo preclinical evaluation.
  • [MeSH-major] Boronic Acids / therapeutic use. Killer Cells, Natural / pathology. Leukemia, T-Cell / drug therapy. Lymphoma, T-Cell / drug therapy. Pyrazines / therapeutic use
  • [MeSH-minor] Apoptosis / drug effects. Bortezomib. Cell Line, Tumor. Drug Screening Assays, Antitumor. Humans. Protease Inhibitors / therapeutic use. Tumor Cells, Cultured

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  • (PMID = 17579189.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Letter; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Boronic Acids; 0 / Protease Inhibitors; 0 / Pyrazines; 69G8BD63PP / Bortezomib
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38. Jeon YK, Park CH, Kim KY, Li YC, Kim J, Kim YA, Paik JH, Park BK, Kim CW, Kim YN: The heat-shock protein 90 inhibitor, geldanamycin, induces apoptotic cell death in Epstein-Barr virus-positive NK/T-cell lymphoma by Akt down-regulation. J Pathol; 2007 Oct;213(2):170-9
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  • [Title] The heat-shock protein 90 inhibitor, geldanamycin, induces apoptotic cell death in Epstein-Barr virus-positive NK/T-cell lymphoma by Akt down-regulation.
  • NK/T-cell lymphoma (NKTL) is strongly associated with latent Epstein-Barr virus (EBV) infection.
  • Recently, latent membrane protein 1 (LMP1), an EBV oncoprotein, was reported to activate the phosphatidylinositol-3 kinase (PI3K)/Akt pathway for cell survival.
  • Because geldanamycin (GA) and its derivative, 17-allylamino-17-demethoxygeldanamycin (17-AAG), exhibit anti-tumour activity by degrading HSP90 client proteins, including Akt, we investigated the effect of GA and 17-AAG on the survival of NKTL cell lines.
  • EBV-positive NKTL cell lines, Hank-1 and NK-YS, and an EBV-negative NK leukaemia cell line, NK-L, were treated with PI3K and Akt inhibitors, GA, and 17-AAG, and were subjected to apoptosis and cell viability assays, and immunoblot analysis.
  • EBV-positive B-lymphoblastoid cell lines IM9 and LMP1-transfected IM9 (IM9-LMP1) were also included.
  • Hank-1 and NK-YS cell viability was compromised and apoptosis was induced by LY294002 (PI3K inhibitor) or Akt inhibitor II.
  • The intrinsic level of pAkt was higher in EBV-positive NKTL cells than in EBV-negative NK-L, and GA or 17-AAG decreased the viability of NKTL cells more efficiently than NK-L.
  • Importantly, GA showed little effect on the viability of normal peripheral NK cells as non-neoplastic counterparts for comparison.
  • [MeSH-major] Antibiotics, Antineoplastic / pharmacology. Apoptosis / drug effects. Benzoquinones / pharmacology. HSP90 Heat-Shock Proteins / antagonists & inhibitors. Herpesvirus 4, Human / isolation & purification. Lactams, Macrocyclic / pharmacology. Lymphoma, Extranodal NK-T-Cell / pathology
  • [MeSH-minor] Cell Survival. Down-Regulation / drug effects. Drug Evaluation, Preclinical. Humans. Lymphoma, B-Cell / metabolism. Lymphoma, B-Cell / pathology. Lymphoma, B-Cell / virology. Membrane Potential, Mitochondrial / physiology. Oncogene Protein v-akt / metabolism. Phosphatidylinositol 3-Kinases / metabolism. Signal Transduction. Tumor Cells, Cultured

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  • (PMID = 17768706.001).
  • [ISSN] 0022-3417
  • [Journal-full-title] The Journal of pathology
  • [ISO-abbreviation] J. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Benzoquinones; 0 / HSP90 Heat-Shock Proteins; 0 / Lactams, Macrocyclic; 4GY0AVT3L4 / tanespimycin; EC 2.7.1.- / Phosphatidylinositol 3-Kinases; EC 2.7.11.1 / Oncogene Protein v-akt; Z3K3VJ16KU / geldanamycin
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39. Le Gouill S, Milpied N, Buzyn A, De Latour RP, Vernant JP, Mohty M, Moles MP, Bouabdallah K, Bulabois CE, Dupuis J, Rio B, Gratecos N, Yakoub-Agha I, Attal M, Tournilhac O, Decaudin D, Bourhis JH, Blaise D, Volteau C, Michallet M, Société Française de Greffe de Moëlle et de Thérapie Cellulaire: Graft-versus-lymphoma effect for aggressive T-cell lymphomas in adults: a study by the Société Francaise de Greffe de Moëlle et de Thérapie Cellulaire. J Clin Oncol; 2008 May 10;26(14):2264-71
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  • [Title] Graft-versus-lymphoma effect for aggressive T-cell lymphomas in adults: a study by the Société Francaise de Greffe de Moëlle et de Thérapie Cellulaire.
  • PURPOSE: Aggressive T-cell lymphomas (ATCLs) represent 10% to 15% of non-Hodgkin's lymphomas (NHLs) in adults.
  • ATCLs show a worse prognosis than B-cell lymphomas.
  • PATIENTS AND METHODS: On behalf of the Société Française de Greffe de Moëlle et de Thérapie Cellulaire, we conducted a retrospective analysis including 77 ATCL patients who underwent allogeneic stem-cell transplantation (alloSCT).
  • RESULTS: The different diagnosis included anaplastic large-cell lymphoma (ALCL; n = 27), peripheral T-cell lymphoma not otherwise specified (PTCL-NOS; n = 27), angioimmunoblastic T-cell lymphoma (AITL; n = 11), hepatosplenic gamma/delta lymphoma (HSL; n = 3), T-cell granular lymphocytic leukemia (T-GLL; n = 1), nasal natural killer (NK)/T-cell lymphoma (nasal-NK/L; n = 3) or non-nasal NK/T-cell lymphoma (non-nasal-NK/L; n = 2), enteropathy-type T-cell (n = 1), and human T-lymphotropic virus (HTLV)-1 lymphoma (n = 2).
  • CONCLUSION: We conclude that alloSCT is a potentially efficient therapy for NK/T lymphomas and is worth further investigation through prospective clinical trials.
  • [MeSH-major] Graft vs Tumor Effect / immunology. Lymphoma, T-Cell / immunology. Lymphoma, T-Cell / therapy. Stem Cell Transplantation


40. Wong T, Ko JY, Wang FS, Chen YJ, Ma MC: Epstein-Barr virus associated extranodal natural killer T cell lymphoma of nasal type mimicking pyogenic osteomyelitis of the proximal humerus. Chang Gung Med J; 2008 May-Jun;31(3):314-9
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  • [Title] Epstein-Barr virus associated extranodal natural killer T cell lymphoma of nasal type mimicking pyogenic osteomyelitis of the proximal humerus.
  • Extranodal natural killer (NK) cell lymphoma/leukemia, nasal type, is rare but highly aggressive.
  • We report a male patient who suffered from EBV associated NK/T cell lymphoma of the proximal humerus but presented as pyogenic osteomyelitis with the clinical signs and symptoms of fever, local erythema, elevated erythrocyte sedimation rate and C-reactive protein.
  • The patient developed tumor recurrence and died due to pneumonia and respiratory failure 10 months after the initial diagnosis.
  • [MeSH-major] Epstein-Barr Virus Infections / complications. Humerus. Lymphoma, Extranodal NK-T-Cell / diagnosis. Osteomyelitis / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male. Suppuration

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  • (PMID = 18782956.001).
  • [ISSN] 2072-0939
  • [Journal-full-title] Chang Gung medical journal
  • [ISO-abbreviation] Chang Gung Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China (Republic : 1949- )
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41. Kim D, Ko Y, Suh Y, Koo H, Huh J, Lee W: Characteristics of Epstein-Barr virus associated childhood non-Hodgkin's lymphoma in the Republic of Korea. Virchows Arch; 2005 Sep;447(3):593-6
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  • [Title] Characteristics of Epstein-Barr virus associated childhood non-Hodgkin's lymphoma in the Republic of Korea.
  • To analyze the clinicopathologic characteristics of childhood non-Hodgkin's lymphoma (NHL) associated with Epstein-Barr virus (EBV), EBER in situ hybridization was performed in 80 cases of NHLs.
  • EBER-positive lymphomas account for 25% (20/80) and include NK/T-cell lymphoma (6/6), aggressive NK-cell leukemia (1/1), peripheral T cell lymphoma (5/11), diffuse large B-cell lymphoma (5/14), hydroa-like T-cell lymphoma (1/1), marginal zone B-cell lymphoma (1/2), and post-transplantation lymphoproliferative disorder (1/1).
  • Other types including 19 cases of Burkitt's lymphoma were negative.
  • Clinically, patients with EBV-positive B-cell lymphomas were cured with chemotherapy, whereas EBV-associated NK- and T cell lymphomas pursued fatal clinical course.
  • In conclusion, EBVs infected in childhood NHLs are frequently associated not only with NK- and T- cell lymphomas but also large B-cell lymphomas.
  • [MeSH-major] Epstein-Barr Virus Infections / epidemiology. Lymphoma, Non-Hodgkin / virology. Tumor Virus Infections / epidemiology

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  • (PMID = 15991005.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / EBNA-2 protein, Human herpesvirus 4; 0 / EBV-associated membrane antigen, Epstein-Barr virus; 0 / Epstein-Barr Virus Nuclear Antigens; 0 / RNA-Binding Proteins; 0 / Ribosomal Proteins; 0 / Viral Matrix Proteins; 0 / Viral Proteins; 135844-68-7 / RPL22 protein, human
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42. Lee AJ, Kim SG, Jeon CH, Suh HS, Yoon GS, Seo AN: A case of natural killer cell leukemia misdiagnosed as tuberculous lymphadenopathy. Korean J Lab Med; 2009 Jun;29(3):194-8
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  • [Title] A case of natural killer cell leukemia misdiagnosed as tuberculous lymphadenopathy.
  • Natural killer (NK) cell neoplasms are a group of rare but highly malignant tumors.
  • We report here one case of NK cell leukemia.
  • Because of the absence of the markers of T-cell, B-cell, and myeloid lineage-specific antigens, we added CD16/56 for the immunophenotyping and the blasts were positive (94%).
  • The tumor cells of biopsied lymph node were only positive for CD56, consistent with NK cell lymphoma.
  • Thus this patient was diagnosed with blastic NK cell lymphoma/leukemia involving bone marrow and lymph node.
  • [MeSH-major] Killer Cells, Natural / pathology. Leukemia / diagnosis
  • [MeSH-minor] Antigens, CD45 / metabolism. Bone Marrow / pathology. Female. HLA-DR Antigens / metabolism. Humans. Middle Aged. Tuberculosis, Lymph Node / diagnosis

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  • (PMID = 19571615.001).
  • [ISSN] 1598-6535
  • [Journal-full-title] The Korean journal of laboratory medicine
  • [ISO-abbreviation] Korean J Lab Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / HLA-DR Antigens; EC 3.1.3.48 / Antigens, CD45
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43. Nadella MV, Kisseberth WC, Nadella KS, Thudi NK, Thamm DH, McNiel EA, Yilmaz A, Boris-Lawrie K, Rosol TJ: NOD/SCID mouse model of canine T-cell lymphoma with humoral hypercalcaemia of malignancy: cytokine gene expression profiling and in vivo bioluminescent imaging. Vet Comp Oncol; 2008 Mar;6(1):39-54
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  • [Title] NOD/SCID mouse model of canine T-cell lymphoma with humoral hypercalcaemia of malignancy: cytokine gene expression profiling and in vivo bioluminescent imaging.
  • Lymphoma is a malignant neoplasm arising from B or T lymphocytes.
  • In dogs, one-third of lymphomas are highly aggressive multicentric T-cell lymphomas that are often associated with humoral hypercalcaemia of malignancy (HHM).
  • There are no cell lines or animal models to investigate the pathogenesis of T-cell lymphoma and HHM in dogs.
  • We developed the first xenograft model by injecting lymphoma cells from an Irish Wolfhound intraperitoneally into NOD/SCID mice.
  • The mice developed multicentric lymphoma along with HHM and increased parathyroid hormone-related protein (PTHrP) as occurs in dogs with T-cell lymphoma.
  • Using cytokine complementary DNA arrays, we identified genes that have potential implications in the pathogenesis of T-cell lymphoma.
  • Quantitative reverse transcriptase-polymerase chain reaction (RT-PCR) of T-cell lymphoma samples from hypercalcaemic canine patients showed that PTHrP likely plays a central role in the pathogenesis of HHM and that hypercalcaemia is the result of a combinatorial effect of different hypercalcaemic factors.
  • Finally, we monitored in vivo tumour progression and metastases in the mouse model by transducing the lymphoma cells with a lentiviral vector that encodes a luciferase-yellow fluorescent protein reporter and showed that in vivo trafficking patterns in this model were similar to those seen in dogs.
  • This unique mouse model will be useful for translational research in lymphoma and for investigating the pathogenesis of T-cell lymphoma and HHM in the dog.

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  • (PMID = 19178662.001).
  • [ISSN] 1476-5829
  • [Journal-full-title] Veterinary and comparative oncology
  • [ISO-abbreviation] Vet Comp Oncol
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA100730; None / None / / P01 CA100730-01; United States / NCI NIH HHS / CA / CA 100730; United States / NCI NIH HHS / CA / P01 CA100730-01
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Cytokines; 0 / Parathyroid Hormone-Related Protein; EC 1.13.12.- / Luciferases; SY7Q814VUP / Calcium
  • [Other-IDs] NLM/ NIHMS175837; NLM/ PMC2832741
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44. Gill H, Liang RH, Tse E: Extranodal natural-killer/t-cell lymphoma, nasal type. Adv Hematol; 2010;2010:627401
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  • [Title] Extranodal natural-killer/t-cell lymphoma, nasal type.
  • The World Health Organization (WHO) classification recognizes 2 main categories of natural killer (NK) cell-derived neoplasms, namely, extranodal NK/T-cell lymphoma, nasal type, and aggressive NK-cell leukaemia.
  • Extranodal nasal NK/T-cell lymphoma is more frequent in the Far East and Latin America.
  • The role of autologous hematopoietic stem cell transplantation is yet to be clearly defined.
  • Allogeneic hematopoietic stem cell transplantation, with the putative graft-versus-lymphoma effect, offers a potentially curative option in patients with advanced disease.

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  • (PMID = 21234094.001).
  • [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/ PMC3018635
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45. Kim SH, Ko WT, Suh MK, Ha GY, Kim JR: A Case of Aggressive NK/T-cell Lymphoma/Leukemia with Cutaneous Involvement in Adolescence. Ann Dermatol; 2008 Jun;20(2):77-81
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  • [Title] A Case of Aggressive NK/T-cell Lymphoma/Leukemia with Cutaneous Involvement in Adolescence.
  • NK/T-cell lymphoma (NKTCL) is characterized by the expression of the NK-cell antigen CD56.
  • Non-nasal NK/T-cell lymphomas are subdivided into primary cutaneous and 4 subtypes of secondary cutaneous lymphomas; nasal type, aggressive, blastic (blastoid), and other specific NK-like cell lymphoma.
  • Aggressive NK/T-cell lymphoma/leukemia is a rare leukemic variant of nasal type NKTCL.
  • We herein report a rare case of aggressive NK/T-cell lymphoma/leukemia with cutaneous involvement in adolescence.

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  • (PMID = 27303165.001).
  • [ISSN] 1013-9087
  • [Journal-full-title] Annals of dermatology
  • [ISO-abbreviation] Ann Dermatol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC4904057
  • [Keywords] NOTNLM ; Adolescence / Aggressive NK/T-cell lymphoma/leukemia
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46. Warnnissorn N, Kanitsap N, Kulkantrakorn K, Assanasen T: Natural killer cell malignancy associated with Epstein-Barr virus and hemophagocytic syndrome. J Med Assoc Thai; 2007 May;90(5):982-7
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  • [Title] Natural killer cell malignancy associated with Epstein-Barr virus and hemophagocytic syndrome.
  • Natural killer cell malignancy is a rare and aggressive lymphoid neoplasm encompassing extra-nodal NK/T-cell lymphoma, nasal-type (ENKLN) and aggressive NK-cell lymphoma/leukemia (ANKL).
  • Ancillary techniques studied on paraffin embedded tissues of both cases demonstrated that the neoplastic cells exhibit cytoplasmic CD3+, CD56+ and cytotoxic granules + by immunohistochemistry, absence of T cell receptor gene rearrangement by PCR, and presence of Epstein-Barr virus mRNA (EBER) transcripts by in situ hybridization.
  • The authors reviewed the literature on natural killer cell neoplasm and compared the clinical characteristics, natural history, and association of Epstein-Barr virus infection with hemophagocytic syndrome.
  • [MeSH-major] Epstein-Barr Virus Infections / physiopathology. Killer Cells, Natural / pathology. Leukemia / pathology. Lymphohistiocytosis, Hemophagocytic / physiopathology. Lymphoma / pathology


47. Yamaguchi M, Suzuki R, Kwong YL, Kim WS, Hasegawa Y, Izutsu K, Suzumiya J, Okamura T, Nakamura S, Kawa K, Oshimi K: Phase I study of dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide (SMILE) chemotherapy for advanced-stage, relapsed or refractory extranodal natural killer (NK)/T-cell lymphoma and leukemia. Cancer Sci; 2008 May;99(5):1016-20
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  • [Title] Phase I study of dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide (SMILE) chemotherapy for advanced-stage, relapsed or refractory extranodal natural killer (NK)/T-cell lymphoma and leukemia.
  • Extranodal natural killer (NK)/T-cell lymphoma, nasal type, and aggressive NK-cell leukemia are rare, and their standard therapy has not been established.
  • At level 1, six patients with extranodal NK/T-cell lymphoma, nasal type, were enrolled.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Leukemia, Lymphoid / drug therapy. Lymphoma, Extranodal NK-T-Cell / drug therapy

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  • (PMID = 18294294.001).
  • [ISSN] 1349-7006
  • [Journal-full-title] Cancer science
  • [ISO-abbreviation] Cancer Sci.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; 7S5I7G3JQL / Dexamethasone; EC 3.5.1.1 / Asparaginase; UM20QQM95Y / Ifosfamide; YL5FZ2Y5U1 / Methotrexate
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48. Yonescu R, Hristov AC, Ahmad A, Overby A, Thomas GH, Griffin CA: Cytogenetic characterization of natural killer cell leukemia. Cancer Genet Cytogenet; 2008 Jun;183(2):125-30
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  • [Title] Cytogenetic characterization of natural killer cell leukemia.
  • Natural killer (NK) cell neoplasms are rare neoplasms characterized by cells with NK characteristics.
  • Karyotype of the unstimulated peripheral blood at diagnosis was 46,XX,i(7)(q10),der(17)t(1;17)(q21;p11.1),-18,+mar[15].
  • Notably, a single cell with only an i(7q) was found, suggesting that this was the primary chromosomal abnormality in the neoplasm.
  • The abnormalities seen in chromosomes 7 and 17 are consistent with previous reports of chromosomal abnormalities in NK-cell lymphomas.
  • [MeSH-major] Chromosome Aberrations. Killer Cells, Natural / immunology. Leukemia / genetics

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  • [Copyright] (c) 2008 Elsevier Inc.
  • (PMID = 18503833.001).
  • [ISSN] 1873-4456
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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49. Kwong YL: Hematopoietic stem cell transplantation in natural killer cell lymphoma and leukemia. Int J Hematol; 2010 Dec;92(5):702-7
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  • [Title] Hematopoietic stem cell transplantation in natural killer cell lymphoma and leukemia.
  • Natural killer (NK) cell lymphomas and leukemias are aggressive neoplasms.
  • Clinically, they can be classified into nasal, non-nasal and lymphoma/leukemia subtypes.
  • High-dose chemotherapy and hematopoietic stem cell transplantation (HSCT) may improve patient outcome.
  • For autologous HSCT, a critical review of the literature shows that most patients with nasal NK cell lymphoma in complete remission (CR) appear to do well without HSCT.
  • Therefore, identification of patients with nasal NK cell lymphoma in CR who are at high risk of relapse may be necessary before autologous HSCT can be recommended.
  • Patients with disseminated nasal NK cell lymphoma, non-nasal NK cell lymphoma and NK cell leukemia have poor outcome with autologous HSCT.
  • Continuous efforts should be devoted to risk stratification for identifying high-risk individuals for HSCT, and defining the optimal conditioning regimen for NK cell lymphomas.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation. Killer Cells, Natural / pathology. Leukemia / therapy. Lymphoma / therapy

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  • (PMID = 21107769.001).
  • [ISSN] 1865-3774
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Japan
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50. Suzuki S, Uozumi K, Utsunomiya A, Ishitsuka K, Masamoto I, Owatari S, Makino T, White Y, Arima N: Aggressive NK cell leukaemia after splenectomy: association with CD95-resistant memory T-cell proliferation and recalcitrant clinical course of haemophagocytic syndrome. Eur J Haematol; 2008 Sep;81(3):236-41
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  • [Title] Aggressive NK cell leukaemia after splenectomy: association with CD95-resistant memory T-cell proliferation and recalcitrant clinical course of haemophagocytic syndrome.
  • We describe a 44-yr-old Japanese woman with persistent polyclonal T-cell proliferation and recalcitrant clinical course of haemophagocytic syndrome (HPS).
  • T cells bearing alphabeta T-cell receptors (TCR) expressed increased amounts of CD95 and of CD45RO, which are phenotypically memory T cells.
  • Aggressive natural killer cell leukaemia (ANKL) without an association with Epstein-Barr virus was detected 1 month after therapeutic splenectomy that followed 3 yr of immunosuppressive therapy against HPS.
  • The immunophenotype of these leukaemia cells was CD56, CD16(dim), CD7, CD45RA and they expressed some CD2, CD8 and HLA-DR.
  • Latent NK-cell malignancy seemed to cause the CD95-resistant memory T-cell proliferation and splenectomy resulted in overt ANKL progression.
  • There should be careful consideration of the risks versus benefits of splenectomy in HPS, in light of the possibility of fatal leukaemia/lymphoma progression.
  • [MeSH-major] Antigens, CD95 / physiology. Killer Cells, Natural / immunology. Leukemia / immunology. Leukemia / therapy. Lymphohistiocytosis, Hemophagocytic / immunology. Lymphohistiocytosis, Hemophagocytic / therapy. Splenectomy / adverse effects
  • [MeSH-minor] Adult. Cell Proliferation. Chromosome Aberrations. Disease Progression. Fatal Outcome. Female. Flow Cytometry. Follow-Up Studies. Humans. Immunophenotyping. Immunosuppressive Agents / therapeutic use. Karyotyping. Risk Factors

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  • (PMID = 18510705.001).
  • [ISSN] 1600-0609
  • [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 / Antigens, CD95; 0 / Immunosuppressive Agents
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51. Murashige N, Kami M, Kishi Y, Kim SW, Takeuchi M, Matsue K, Kanda Y, Hirokawa M, Kawabata Y, Matsumura T, Kusumi E, Hirabayashi N, Nagafuji K, Suzuki R, Takeuchi K, Oshimi K: Allogeneic haematopoietic stem cell transplantation as a promising treatment for natural killer-cell neoplasms. Br J Haematol; 2005 Aug;130(4):561-7
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  • [Title] Allogeneic haematopoietic stem cell transplantation as a promising treatment for natural killer-cell neoplasms.
  • The efficacy of allogeneic haematopoietic stem-cell transplantation (allo-HSCT) for natural killer (NK)-cell neoplasms is unknown.
  • We investigated the results of allo-HSCT for NK-cell neoplasms between 1990 and 2003 through questionnaires.
  • After reclassification by a haematopathologist, of 345 patients who underwent allo-HSCT for malignant lymphoma, 28 had NK-cell neoplasms (World Health Organization classification): extranodal NK/T-cell lymphoma (n=22), blastic NK-cell lymphoma (n=3), and aggressive NK-cell leukaemia (n=3).
  • Stem-cell source was bone marrow in eight and mobilised peripheral blood in 20.
  • In multivariate analysis, stem cell source (BM versus peripheral blood; relative risk 3.03), age (>or=40 years vs. <40 years; relative risk 2.85), and diagnoses (extranodal NK/T-cell lymphoma versus others; relative risk 3.94) significantly affected PFS.
  • Allo-HSCT is a promising treatment for NK-cell neoplasms.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation. Killer Cells, Natural / immunology. Lymphoma, T-Cell / immunology
  • [MeSH-minor] Adult. Bone Marrow Transplantation. Disease-Free Survival. Female. Follow-Up Studies. Graft vs Host Disease. Humans. Male. Middle Aged. Peripheral Blood Stem Cell Transplantation. Proportional Hazards Models. Risk. Transplantation, Homologous

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  • (PMID = 16098071.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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52. Chee LC, Fahey V, Juneja S, Lieschke GJ, Szer J: Images in haematology. Relapsed blastic natural killer cell leukaemia with splenic rupture. Br J Haematol; 2006 Oct;135(1):2
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  • [Title] Images in haematology. Relapsed blastic natural killer cell leukaemia with splenic rupture.
  • [MeSH-major] Killer Cells, Natural. Lymphoma, T-Cell / complications. Splenic Rupture / etiology

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  • (PMID = 16848796.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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53. Huang Q, Chang KL, Gaal KK, Weiss LM: An aggressive extranodal NK-cell lymphoma arising from indolent NK-cell lymphoproliferative disorder. Am J Surg Pathol; 2005 Nov;29(11):1540-3
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  • [Title] An aggressive extranodal NK-cell lymphoma arising from indolent NK-cell lymphoproliferative disorder.
  • Indolent NK-cell lymphoproliferative disorder, also known as chronic natural killer (NK) cell large granular lymphocytosis (leukemia), is a very rare entity in the World Health Organization (WHO) Classification of Tumors of Hematopoietic & Lymphoid Tissues.
  • Unlike aggressive NK-cell leukemia, which is malignant, the WHO does not specify whether indolent NK-cell lymphoproliferative disorder is reactive or neoplastic.
  • Patients with indolent NK-cell lymphoproliferative disorder are usually asymptomatic older adults who have a nonprogressive, very stable clinical course.
  • We report an unusual case of an aggressive extranodal NK-cell lymphoma, non-nasal type, which presented as a subcutaneous tissue mass, which apparently transformed from a preexisting, untreated indolent NK-cell lymphoproliferative disorder in an 65-year-old otherwise healthy white man.
  • The extranodal NK-cell lymphoma and the NK-cell lymphoproliferative disorder in the blood and bone marrow share a distinctive and identical NK-cell immunophenotype and genotype: CD56/CD8/TIA-1-positive and surface CD3-negative, negative for Epstein-Barr virus infection, and no evidence of T-cell and B-cell receptor gene rearrangements.
  • To the best of our knowledge, such aggressive lymphomatous transformation in an indolent NK-cell lymphoproliferative disorder has not been previously reported.
  • [MeSH-major] Killer Cells, Natural / pathology. Lymphoma, Large B-Cell, Diffuse / pathology. Lymphoproliferative Disorders / complications

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  • (PMID = 16224224.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; VB0R961HZT / Prednisone; CHOP protocol
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54. Souabni A, Jochum W, Busslinger M: Oncogenic role of Pax5 in the T-lymphoid lineage upon ectopic expression from the immunoglobulin heavy-chain locus. Blood; 2007 Jan 1;109(1):281-9
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  • The B-lymphoidPAX5 gene participates in the generation of the t(9;14)(p13;q32) translocation in germinal center B cells, which leads to deregulated PAX5 expression under the control of the immunoglobulin heavy-chain (IgH) locus in a subset of B-cell non-Hodgkin lymphomas.
  • The IgH(P5ki) allele, which corresponds to a germline rather than somatic mutation, is activated in multipotent hematopoietic progenitors and is subsequently expressed in dendritic cells (DCs) and in natural killer (NK), T, and B cells.
  • Ectopic Pax5 expression interferes with normal T-cell development and causes immature T-lymphoblastic lymphomas in IgH(P5ki/+) and IgH(P5ki/P5ki) mice.
  • Aggressive T-cell lymphomas develop even faster in Ik(Pax5/+) mice expressing Pax5 from the Ikaros locus.
  • Pax5 expression in thymocytes activates B-cell-specific genes and represses T-lymphoid genes, suggesting that Pax5 contributes to lymphomagenesis by deregulating the T-cell gene-expression program.
  • [MeSH-major] B-Cell-Specific Activator Protein / physiology. Cell Differentiation / genetics. Cell Transformation, Neoplastic / genetics. Chromosomes, Human, Pair 14 / genetics. Chromosomes, Human, Pair 9 / genetics. Immunoglobulin Heavy Chains / genetics. Neoplastic Stem Cells / pathology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics. Promoter Regions, Genetic. T-Lymphocytes / pathology. Translocation, Genetic
  • [MeSH-minor] Alleles. Animals. B-Lymphocytes / metabolism. B-Lymphocytes / pathology. Bone Marrow Transplantation. Cell Lineage. Dendritic Cells / metabolism. Dendritic Cells / pathology. Embryonal Carcinoma Stem Cells. Gene Expression Regulation, Neoplastic. Humans. Ikaros Transcription Factor / genetics. Killer Cells, Natural / metabolism. Killer Cells, Natural / pathology. Lymphocytes / metabolism. Lymphocytes / pathology. Lymphoma, B-Cell / genetics. Lymphoma, B-Cell / pathology. Mice. Mice, Inbred C57BL. Mutagenesis, Insertional. Neoplasm Transplantation. Radiation Chimera

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  • (PMID = 16968900.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / B-Cell-Specific Activator Protein; 0 / Immunoglobulin Heavy Chains; 0 / PAX5 protein, human; 0 / Pax5 protein, mouse; 0 / Zfpn1a1 protein, mouse; 148971-36-2 / Ikaros Transcription Factor
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55. Armor JF, Fazili J, Toubia N, Kern W, Kamble R, Kharfan-Dabaja MA: Remission of natural-killer cell lymphoma of the liver with anti-hepatitis C therapy. Am J Hematol; 2005 Mar;78(3):212-5
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  • [Title] Remission of natural-killer cell lymphoma of the liver with anti-hepatitis C therapy.
  • A rare subtype of malignancy arising from cells of putative natural killer (NK) origin is being recognized as a distinct clinicopathological entity.
  • Viruses including hepatitis C have been reported in association with various types of NHL but not the NK-cell subtype.
  • We hereby report a unique case of a patient with hepatitis C who developed hepatic NK-cell lymphoma and chronic NK-cell leukemia.
  • [MeSH-major] Antiviral Agents / therapeutic use. Hepatitis C / drug therapy. Killer Cells, Natural / pathology. Liver Neoplasms / pathology. Lymphoma, Non-Hodgkin / pathology. Tumor Virus Infections / pathology

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  • (PMID = 15726605.001).
  • [ISSN] 0361-8609
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antiviral Agents; 0 / Interferon-alpha; 0 / Recombinant Proteins; 49717AWG6K / Ribavirin; 99210-65-8 / interferon alfa-2b
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56. Löffler H, Kosely F, Ho AD, Krämer A: [Blastic plasmacytoid dendritic cell neoplasm - a rare differential diagnosis of neoplastic skin infiltrations associated with systemic symptoms]. Dtsch Med Wochenschr; 2009 Sep;134(39):1927-30
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  • [Title] [Blastic plasmacytoid dendritic cell neoplasm - a rare differential diagnosis of neoplastic skin infiltrations associated with systemic symptoms].
  • A skin biopsy was suggestive of malignant lymphoma of the skin.
  • Additional immunohistochemistry of the skin specimen as well as cytologic and flow cytometric examination of the bone marrow revealed an immature cell population expressing CD4 and CD56 which infiltrated both the dermis and, with an infiltration grade of about 85 %, the bone marrow.
  • DIAGNOSIS: Blastic plasmacytoid dendritic cell neoplasm (formerly known as blastic NK cell lymphoma).
  • CONCLUSION: The blastic plasmacytoid dendritic cell neoplasm is a rare, aggressive hematopoietic neoplasm most likely related to acute myeloid leukemia (AML).
  • Since cutaneous involvement is regularly present at diagnosis, the differential diagnosis of unexplained skin lesions should include this disease entity, especially if peripheral blood abnormalities are present.
  • [MeSH-major] Leukemia, Myeloid, Acute / diagnosis. Skin / pathology. Skin Neoplasms / diagnosis
  • [MeSH-minor] Aged. Antineoplastic Agents / therapeutic use. Biopsy. Bone Marrow / pathology. Diagnosis, Differential. Exanthema. Female. Flow Cytometry. Humans. Immunohistochemistry. Immunophenotyping. Pancytopenia. Prognosis. Remission Induction

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  • [Copyright] Georg Thieme Verlag KG Stuttgart, New York.
  • (PMID = 19760552.001).
  • [ISSN] 1439-4413
  • [Journal-full-title] Deutsche medizinische Wochenschrift (1946)
  • [ISO-abbreviation] Dtsch. Med. Wochenschr.
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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57. Coppo P, Gouilleux-Gruart V, Huang Y, Bouhlal H, Bouamar H, Bouchet S, Perrot C, Vieillard V, Dartigues P, Gaulard P, Agbalika F, Douay L, Lassoued K, Gorin NC: STAT3 transcription factor is constitutively activated and is oncogenic in nasal-type NK/T-cell lymphoma. Leukemia; 2009 Sep;23(9):1667-78
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  • [Title] STAT3 transcription factor is constitutively activated and is oncogenic in nasal-type NK/T-cell lymphoma.
  • Nasal-type natural killer (NK) cell lymphoma is an infrequent aggressive malignant disease with very poor prognosis.
  • For this, we established and characterized a continuous interleukin 2-dependent NK cell line (MEC04) from a patient with a fatal nasal-type NK-cell lymphoma.
  • By using recombinant transducible TAT-STAT3-beta (beta isoform), TAT-STAT3Y705F (a STAT3 protein mutated on Y705 residue, which prevents STAT3 dimerization) and peptides inhibiting specifically STAT3 dimerization, we inhibited STAT3 phosphorylation and cell growth, with cell death induction.
  • Finally, STAT3 was phosphorylated in Y705 residue in the nuclei of lymphoma cells in eight/nine patients with nasal-type NK/T-cell lymphoma and in YT, another NK cell line.
  • Our results suggest that STAT3 protein has a major role in the oncogenic process of nasal-type NK-cell lymphomas, and may represent a promising therapeutical target.
  • [MeSH-major] Killer Cells, Natural / pathology. Lymphoma, T-Cell / etiology. Nose Neoplasms / etiology. STAT3 Transcription Factor / physiology

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  • (PMID = 19421230.001).
  • [ISSN] 1476-5551
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / BCL2L1 protein, human; 0 / STAT3 Transcription Factor; 0 / STAT3 protein, human; 0 / bcl-X Protein; 82115-62-6 / Interferon-gamma; EC 2.7.10.2 / JAK2 protein, human; EC 2.7.10.2 / Janus Kinase 2; EC 2.7.11.24 / Extracellular Signal-Regulated MAP Kinases
  • [Other-IDs] NLM/ HALMS419047; NLM/ PMC2796333
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58. Ma Y, Chen B, Xu X, Lin G: Myeloid/natural killer cell precursor acute leukemia with multiple subcutaneous nodules as the initial presentation: a case report and literature review. Int J Hematol; 2009 Sep;90(2):243-7
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  • [Title] Myeloid/natural killer cell precursor acute leukemia with multiple subcutaneous nodules as the initial presentation: a case report and literature review.
  • Myeloid/natural killer (NK) cell precursor acute leukemia is a rare neoplasm, which is characterized by high incidence of extramedullary infiltration, especially in the mediastinum and lymph nodes, an aggressive course and poor prognosis.
  • As coexpressing myeloid and NK-cell antigens, myeloid/NK-cell precursor acute leukemia (MNKL) may pose diagnostic difficulty.
  • Because the developmental pathway of normal NK cells is not well understood, neoplams of NK-cell origin are not clearly identified.
  • A diagnosis of MNKL was finally made by skin biopsy, bone marrow immunohistochemistry and immunophenotypic analysis.
  • [MeSH-major] Killer Cells, Natural / pathology. Leukemia, Myeloid / pathology. Myeloid Cells / pathology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology. Skin / pathology
  • [MeSH-minor] Bone Marrow / pathology. Diagnosis, Differential. Humans. Leukemic Infiltration. Male. Young Adult

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  • (PMID = 19639272.001).
  • [ISSN] 1865-3774
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 34
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59. Sakata K, Someya M, Omatsu M, Asanuma H, Hasegawa T, Ichimiya S, Hareyama M, Himi T: The enhanced expression of the matrix metalloproteinase 9 in nasal NK/T-cell lymphoma. BMC Cancer; 2007;7:229
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  • [Title] The enhanced expression of the matrix metalloproteinase 9 in nasal NK/T-cell lymphoma.
  • BACKGROUND: Nasal NK/T cell lymphoma is an aggressive disease and has a poor prognosis.
  • Nasal NK/T cell lymphoma is refractory to conventional chemotherapy and has strong tendency of widespread relapse or dissemination into distant sites.
  • METHODS: We immunohistochemically studied nasal NK/T-cell lymphoma to elucidate the unique characteristics of nasal NK/T-cell lymphoma, such as its higher metastatic tendency and its vast necrosis which leads to destruction of the involved tissues.
  • The expression of P-glycoprotein and MMP-9 was evaluated in the 20 patients with nasal NK/T-cell lymphoma and 25 with nasal non-NK/T-cell lymphoma and the relationship between expression of these proteins and clinical results were analyzed in this report.
  • RESULTS: Overall 5-year survival rates for patients with nasal NK/T cell lymphoma, and nasal non-NK/T cell lymphoma were 51%, and 84%.
  • Distant involvement free 5-year survival rates for patients with nasal NK/T cell lymphoma, and nasal non-NK/T cell lymphoma were 53%, and 79%.
  • Sixteen of the 19 patients with nasal NK/T cell lymphoma expressed MMP-9.
  • In contrast, only 8 of the 22 patients with nasal non-NK/T cell lymphoma expressed MMP-9.
  • CONCLUSION: Positive immunoreactivity for P-glycoprotein was not an independent prognostic factor in nasal NK/T-cell lymphomas, which stresses the importance of exploring other mechanisms of drug resistance.
  • The strong expression of MMP-9 is uniquely characteristic of nasal NK/T cell lymphoma and may contribute to its strong tendency to disseminatate and the extensive necrosis which is always seen.
  • [MeSH-major] Lymphoma, Extranodal NK-T-Cell / metabolism. Lymphoma, Extranodal NK-T-Cell / pathology. Matrix Metalloproteinase 9 / metabolism. Nasal Cavity / pathology. Nose Neoplasms / metabolism. Nose Neoplasms / pathology

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  • (PMID = 18093334.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / P-Glycoprotein; EC 3.4.24.35 / Matrix Metalloproteinase 9
  • [Other-IDs] NLM/ PMC2238761
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60. Ma R, Xu YG, Yang XH, Hu XM, Li L, Tang XD, Zhang SS, Xu S, Wang HZ, Liu F: [Immunophenotypic features in leukemia of NK cell series]. Zhongguo Shi Yan Xue Ye Xue Za Zhi; 2006 Feb;14(1):35-8
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  • [Title] [Immunophenotypic features in leukemia of NK cell series].
  • The aim was to investigate the immunophenotypes of NK series leukemia.
  • Immunophenotypes of 297 cases of acute leukemia (AL) were measured by flow cytometry, and these immucopenotypic features were analyzed.
  • 6 cases were NK series leukemia and 37 cases were acute myelogenous leukemia with CD56 expressed.
  • One patient has been diagnosed as myeloid/NK cell precursor acute leukemia, two patients were blastic NK cell leukemia, one was supposed to be NK-like T-cell lymphoma/leukemia, while another one was large granular lymphocyte leukemia (LGLL).
  • It is concluded that almost all of CD56 positive leukemia were acute myelogenous leukemia with CD56 expressed.
  • The immunophenotypes of NK series leukemia were antigens from hematopoietic stem cells to T/NK progenitor cells with meyloid antigen positive, and through NK progenitors to mature NK cells.
  • The immunophenotypes of heterogeneous NK leukemia cells are different, that should be carefully distinguished.

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  • (PMID = 16584587.001).
  • [ISSN] 1009-2137
  • [Journal-full-title] Zhongguo shi yan xue ye xue za zhi
  • [ISO-abbreviation] Zhongguo Shi Yan Xue Ye Xue Za Zhi
  • [Language] CHI
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Antigens, CD56
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61. Kohrt H, Advani R: Extranodal natural killer/T-cell lymphoma: current concepts in biology and treatment. Leuk Lymphoma; 2009 Nov;50(11):1773-84
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  • [Title] Extranodal natural killer/T-cell lymphoma: current concepts in biology and treatment.
  • Natural killer/T-cell (NK/T) lymphomas represent a group of rare tumors of NK and NK-T cells.
  • The World Health Organization classifies NK-cell tumors into three types, extranodal NK/T-cell lymphomas (ENKL, nasal and non-nasal), NK-cell leukemias, and a blastic variant (CD4-positive, CD56-positive hematodermic neoplasms).
  • Though considerable advances have been made in our understanding of NK-cell biology, malignant transformation including the role of Epstein-Barr virus, and prognosis, the rare nature of ENKL and its heterogeneity limit the ability to standardize therapy.
  • The clinical course of advanced disease is highly aggressive with frequent chemotherapy resistance and a poor prognosis.
  • Therapeutic approaches to advanced-stage or relapsed and refractory disease, including the appropriate sequence of chemotherapy, combined modality therapy, and stem cell transplantation is not well-established.
  • International and multicenter clinical trials are needed for this rare and aggressive disease.
  • [MeSH-major] Lymphoma, Extranodal NK-T-Cell / pathology. Lymphoma, Extranodal NK-T-Cell / therapy
  • [MeSH-minor] Combined Modality Therapy. Drug Therapy. Hematopoietic Stem Cell Transplantation. Humans. Neoplasm Staging. Prognosis. Radiotherapy. Survival Analysis

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  • (PMID = 19883307.001).
  • [ISSN] 1029-2403
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Grant] United States / NHLBI NIH HHS / HL / T32 HL007952
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 84
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62. Alekshun TJ, Sokol L: Diseases of large granular lymphocytes. Cancer Control; 2007 Apr;14(2):141-50
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  • [Title] Diseases of large granular lymphocytes.
  • BACKGROUND: Clonal diseases of large granular lymphocytes (LGLs) are rare lymphoproliferative malignancies that arise from either mature T-cell (CD3+) or natural killer (NK)-cell (CD3-) lineages.
  • They manifest a distinct biologic behavior that ranges from indolent to very aggressive.
  • METHODS: We discuss four distinct diseases involving LGLs: indolent T-cell LGL leukemia, aggressive T-cell LGL leukemia, chronic NK-cell leukemia, and aggressive NK-cell leukemia.
  • RESULTS: Sustained LGLs, characteristic immunophenotype, clonal origin of leukemic cells, and clinical presentation are the most important features that distinguish indolent from aggressive subtypes of LGL leukemia and guide the selection of therapy.
  • Patients with symptomatic indolent T-cell or NK-cell LGL leukemia are usually treated with immunosuppressive therapies in contrast to aggressive T-cell and NK-cell LGL leukemia, which require intensive chemotherapy induction regimens.
  • CONCLUSIONS: Future scientific advances focusing on the delineation of molecular pathogenic mechanisms and the development of new targeted therapies for each distinct LGL leukemia entity should lead to improved outcomes of patients with these disorders.
  • [MeSH-major] Antigens, CD3. Killer Cells, Natural / pathology. Leukemia, Lymphoid / pathology. Leukemia, T-Cell / pathology. Lymphocytes / pathology

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  • (PMID = 17387299.001).
  • [ISSN] 1073-2748
  • [Journal-full-title] Cancer control : journal of the Moffitt Cancer Center
  • [ISO-abbreviation] Cancer Control
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD3
  • [Number-of-references] 71
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63. Ishida F, Kwong YL: Diagnosis and management of natural killer-cell malignancies. Expert Rev Hematol; 2010 Oct;3(5):593-602
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  • [Title] Diagnosis and management of natural killer-cell malignancies.
  • Natural killer (NK)-cell malignancies are uncommon neoplasms, which have been referred to as polymorphic reticulosis or angiocentric T-cell lymphomas in the past.
  • In the current WHO classification, they are categorized as extranodal NK/T-cell lymphoma, nasal type and aggressive NK-cell leukemia.
  • NK-cell malignancies show a geographical predilection for Asian and South American populations and are rare in the west.
  • Pathologically, NK-cell lymphomas show a polymorphic neoplastic infiltrate with angioinvasion and angiodestruction.
  • The lymphoma cells are CD2(+), cytoplasmic CD3ε(+) and CD56(+), with germline T-cell receptor gene.
  • Clinically, NK-cell lymphomas can be classified into nasal, non-nasal and aggressive lymphoma/leukemia subtypes.
  • Most nasal NK-cell lymphomas present with stage I/II disease.
  • Chemotherapy is indicated for stage III/IV nasal NK-cell lymphoma, and the non-nasal and aggressive subtypes.
  • High-dose chemotherapy and hematopoietic stem-cell transplantation with autologous or allogeneic hematopoietic stem cells may be beneficial to selected patients.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Asparaginase / administration & dosage. Killer Cells, Natural / drug effects. Leukemia / diagnosis. Leukemia / therapy. Lymphoma, Extranodal NK-T-Cell / diagnosis. Lymphoma, Extranodal NK-T-Cell / therapy
  • [MeSH-minor] Antigens, CD. Asia / epidemiology. Female. Hematopoietic Stem Cell Transplantation. Herpesvirus 4, Human. Humans. Male. Middle Aged. Prognosis. Recurrence. Severity of Illness Index. South America / epidemiology. Transplantation, Autologous

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  • (PMID = 21083476.001).
  • [ISSN] 1747-4094
  • [Journal-full-title] Expert review of hematology
  • [ISO-abbreviation] Expert Rev Hematol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD; EC 3.5.1.1 / Asparaginase
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64. Nikolova M, Guenova M, Taskov H, Marie-Cardine A, Boumsell L, Bensussan A: SC3 monoclonal antibody defines a novel specific human B-cell surface antigen differentially expressed on B-cell leukaemias and lymphomas and involved in the proliferation of normal and malignant B lymphocytes. Cell Immunol; 2005 Jul-Aug;236(1-2):92-100
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  • [Title] SC3 monoclonal antibody defines a novel specific human B-cell surface antigen differentially expressed on B-cell leukaemias and lymphomas and involved in the proliferation of normal and malignant B lymphocytes.
  • The monoclonal antibody SC3 was raised against the NK leukaemia cell line YTindi.
  • It detected a 98-kDa surface antigen with weak expression on a restricted number of leukaemia cell lines under reducing conditions.
  • SC3 mAb labelled 5-10% of normal peripheral blood lymphocytes corresponding almost exclusively to B lymphocytes, and 60-70% of tonsillar B cells.
  • Practically, all B-CLL studied expressed SC3 mAb reactive epitope although with variable intensity, while MCL and PLL were negative.
  • This effect was much weaker with B-CLL cells but was increased after cross-linking with an anti-IgM antibody.
  • The restricted expression pattern combined with molecular weight and functional data indicate that SC3 mAb may detect a novel B-cell antigen mostly expressed by early and naive B cells.
  • Although its expression in B-cell malignancies was not limited to a single differentiation stage, it might confer specific functional characteristics to the positive malignant cells.
  • [MeSH-major] Antigens, Differentiation, B-Lymphocyte / immunology. Antigens, Surface / immunology. B-Lymphocytes / immunology. Leukemia, B-Cell / immunology. Lymphoma, B-Cell / immunology

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  • (PMID = 16197933.001).
  • [ISSN] 0008-8749
  • [Journal-full-title] Cellular immunology
  • [ISO-abbreviation] Cell. Immunol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antigens, Differentiation, B-Lymphocyte; 0 / Antigens, Surface; 0 / Epitopes, B-Lymphocyte
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65. Ohsugi T, Horie R, Kumasaka T, Ishida A, Ishida T, Yamaguchi K, Watanabe T, Umezawa K, Urano T: In vivo antitumor activity of the NF-kappaB inhibitor dehydroxymethylepoxyquinomicin in a mouse model of adult T-cell leukemia. Carcinogenesis; 2005 Aug;26(8):1382-8
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  • [Title] In vivo antitumor activity of the NF-kappaB inhibitor dehydroxymethylepoxyquinomicin in a mouse model of adult T-cell leukemia.
  • Adult T-cell leukemia (ATL) is an aggressive neoplasm caused by human T-cell leukemia virus type I (HTLV-I).
  • Five-week-old SCID mice in which natural killer (NK) cell activity had been eliminated were inoculated intraperitoneally with the HTLV-I-infected cell lines, TL-Om1, MT-1, MT-2 and HUT-102.
  • In contrast, inoculation of mice with MT-2 and HUT-102 cells elicited high mortality, 100% frequency of gross tumor formation and tumor cell infiltration of various organs, all of which were reduced by coadministration of DHMEQ during the inoculation.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Benzamides / pharmacology. Cyclohexanones / pharmacology. Leukemia-Lymphoma, Adult T-Cell / drug therapy. NF-kappa B / antagonists & inhibitors
  • [MeSH-minor] Animals. Apoptosis / drug effects. Base Sequence. Binding Sites. Cell Division / drug effects. Cell Line, Tumor. Consensus Sequence. Disease Models, Animal. Human T-lymphotropic virus 1 / drug effects. Human T-lymphotropic virus 1 / physiology. Humans. Mice. Transplantation, Heterologous

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  • (PMID = 15831528.001).
  • [ISSN] 0143-3334
  • [Journal-full-title] Carcinogenesis
  • [ISO-abbreviation] Carcinogenesis
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Cyclohexanones; 0 / NF-kappa B; 0 / dehydroxymethylepoxyquinomicin
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66. Min HS, Hyun CL, Paik JH, Jeon YK, Choi G, Park SH, Seo JW, Kim CW: An autopsy case of aggressive CD30+ extra-nodal NK/T-cell lymphoma initially manifested with granulomatous myositis. Leuk Lymphoma; 2006 Feb;47(2):347-52
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  • [Title] An autopsy case of aggressive CD30+ extra-nodal NK/T-cell lymphoma initially manifested with granulomatous myositis.
  • This study reports an autopsy case of a 53 year-old male with rapidly progressive extra-nodal NK/T-cell lymphoma accompanied with unusual clinical and pathologic features.
  • He was initially presented with localized swelling and tenderness in the right lower extremity and the biopsy from the calf muscle was interpreted as granulomatous myositis masquerizing lymphoma.
  • The biopsy from erythematous skin lesion of trunk showed infiltration of medium sized atypical lymphoid cells with relatively plump cytoplasm and immunophenotype of CD30+, CD56+/- and surface CD3-, which lead to the diagnosis of CD30+ anaplastic large cell lymphoma.
  • After confirmation of EBV infection, he was finally diagnosed as extra-nodal NK/T-cell lymphoma with peculiar immunophenotype of CD3 dim+ and CD30+.
  • At autopsy, disseminated angiocentric lymphoma was found all over the internal organs including the brain.
  • This case emphasizes that extra-nodal NK/T-cell lymphoma should be considered as a cause of granulomatous myositis and can express CD30 positivity and CD3 weak positivity, which are unusual but rarely predominant feature of NK/T-cell lymphoma.
  • [MeSH-major] Antigens, CD30 / immunology. Granuloma / pathology. Killer Cells, Natural / pathology. Lymphoma, T-Cell / pathology. Myositis / pathology

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  • (PMID = 16321870.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD3; 0 / Antigens, CD30; 0 / CD3 antigen, zeta chain
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67. Choi YL, Park JH, Kim WS, Lee DY, Lee JH, Yang JM, Lee ES: Aggressive NK-cell leukaemia associated with reactive haemophagocytic syndrome. Clin Exp Dermatol; 2006 Jan;31(1):83-5
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  • [Title] Aggressive NK-cell leukaemia associated with reactive haemophagocytic syndrome.
  • We report a case of aggressive NK-cell leukaemia associated with reactive haemophagocytic syndrome in a 29-year-old Korean woman who had several small purpuric patches on both thighs.
  • Laboratory tests revealed pancytopenia and deranged liver function, and atypical lymphocytes containing toxic granules were detected from peripheral blood and bone marrow.
  • [MeSH-major] Killer Cells, Natural / immunology. Leukemia / immunology. Lymphohistiocytosis, Hemophagocytic / immunology. Lymphoma, T-Cell, Cutaneous / immunology

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  • (PMID = 16309492.001).
  • [ISSN] 0307-6938
  • [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, CD
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68. Suzuki R, Suzumiya J, Yamaguchi M, Nakamura S, Kameoka J, Kojima H, Abe M, Kinoshita T, Yoshino T, Iwatsuki K, Kagami Y, Tsuzuki T, Kurokawa M, Ito K, Kawa K, Oshimi K, NK-cell Tumor Study Group: Prognostic factors for mature natural killer (NK) cell neoplasms: aggressive NK cell leukemia and extranodal NK cell lymphoma, nasal type. Ann Oncol; 2010 May;21(5):1032-40
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  • [Title] Prognostic factors for mature natural killer (NK) cell neoplasms: aggressive NK cell leukemia and extranodal NK cell lymphoma, nasal type.
  • BACKGROUND: Patients with natural killer (NK) cell neoplasms, aggressive NK cell leukemia (ANKL) and extranodal NK cell lymphoma, nasal type (ENKL), have poor outcome.
  • The purpose of this study is to draw a prognostic model of total NK cell neoplasms.
  • Using these four variables, an NK prognostic index was successfully constructed.
  • CONCLUSION: The current prognostic model successfully stratified patients with NK cell neoplasms with different outcomes.


69. Osuji N, Matutes E, Morilla A, Del Giudice I, Wotherspoon A, Catovsky D: Prolonged treatment response in aggressive natural killer cell leukemia. Leuk Lymphoma; 2005 May;46(5):757-63
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prolonged treatment response in aggressive natural killer cell leukemia.
  • We describe a case of natural killer (NK) cell leukemia with acute presentation, systemic symptoms and hepatosplenomegaly.
  • The uniform and aberrant phenotype of NK cells with infiltration of bone marrow and spleen was in keeping with a malignant diagnosis.
  • Aggressive presentation was demonstrated by marked constitutional symptoms and significant tumor burden (liver, spleen, blood, bone marrow).
  • We discuss the difficulties in the diagnosis of this condition, explore possible causes of cytopenia(s), and highlight the role of immunosuppression in controlling disease manifestations in large granular lymphocyte proliferative disorders.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Killer Cells, Natural / pathology. Leukemia / therapy

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  • (PMID = 16019515.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Recombinant Proteins; 11096-26-7 / Erythropoietin; 395575MZO7 / Pentostatin; 83HN0GTJ6D / Cyclosporine
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70. Ino K, Masuya M, Nakamori Y, Suzuki K, Mizutani M, Sekine T, Yamaguchi M, Nakase K, Kaida K, Ogawa H, Katayama N: [Aggressive NK-cell leukemia with sustained relapse-free survival after allogeneic peripheral blood stem cell transplantation]. Rinsho Ketsueki; 2010 Apr;51(4):258-63
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  • [Title] [Aggressive NK-cell leukemia with sustained relapse-free survival after allogeneic peripheral blood stem cell transplantation].
  • A bone marrow examination showed several hemophagocytic macrophages, and a diagnosis of hemophagocytic syndrome was made.
  • A repeat bone marrow examination demonstrated that 28.4% of marrow nucleated cells were atypical lymphocytes, which were positive for CD2, CD7, CD16, CD56, and HLA-DR.
  • Clonality of these proliferating NK cells was confirmed by an analysis of EB virus terminal repeat sequence and cytogenetic analysis, and final diagnosis of aggressive NK-cell leukemia was made.
  • He received allogeneic peripheral blood stem cell transplantation from his one locus mismatched son, and is alive with no evidence of disease 20 months after transplantation.
  • [MeSH-major] Killer Cells, Natural. Leukemia, Lymphoid / therapy. Peripheral Blood Stem Cell Transplantation


71. Liang X, Greffe B, Garrington T, Graham DK: Precursor natural killer cell leukemia. Pediatr Blood Cancer; 2008 Apr;50(4):876-8
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  • [Title] Precursor natural killer cell leukemia.
  • Natural killer (NK) cell tumors are a rare and heterogeneous group of disorders.
  • Immature NK cell tumors are less common, and are less recognized and defined than mature NK cell tumors.
  • There is insufficient experience of diagnosis and treatment with immature NK cell tumors, especially in pediatric patients.
  • Here we describe a pediatric patient with precursor NK cell leukemia and review the literature of the previously reported cases in children to further help characterize the diagnosis, treatment, and outcome.
  • [MeSH-major] Killer Cells, Natural / pathology. Leukemia / pathology. Stem Cells / pathology

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 17417789.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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72. Greer JP, Mosse CA: Natural killer-cell neoplasms. Curr Hematol Malig Rep; 2009 Oct;4(4):245-52
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  • [Title] Natural killer-cell neoplasms.
  • The natural killer (NK)-cell neoplasms are rare, representing less than 1% of non-Hodgkin lymphoma, except in Asia and Latin America, where they represent 3% to 6%.
  • NK-cell neoplasms include immature acute leukemias; a blastic NK-cell lymphoma, which is obsolete because of its plasmacytoid dendritic-cell origin; and mature NK neoplasms, comprising extranodal NK/T-cell lymphoma (ENKL), nasal-type; aggressive NK-cell leukemia; and chronic NK-cell lymphoproliferative disorders, which are often reactive.
  • Epstein-Barr virus is usually detected in tumor cells of ENKL and aggressive NK-cell leukemia.
  • The latter two mature NK neoplasms are relatively chemoresistant because of the frequent expression of P-glycoprotein.
  • Stem cell transplantation is recommended for advanced disease, owing to a poor prognosis.
  • [MeSH-major] Killer Cells, Natural / pathology. Leukemia / pathology. Lymphoma / pathology. Lymphoproliferative Disorders / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Cell Lineage. Cell Transformation, Neoplastic. Humans. Stem Cell Transplantation

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  • (PMID = 20425414.001).
  • [ISSN] 1558-822X
  • [Journal-full-title] Current hematologic malignancy reports
  • [ISO-abbreviation] Curr Hematol Malig Rep
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 42
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73. Gandhi J: Natural killer cell leukaemia. BMJ Case Rep; 2009;2009
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  • [Title] Natural killer cell leukaemia.
  • She was initially admitted with sepsis without an obvious cause but with a differential diagnosis of a haematological malignancy.
  • Her admission blood tests showed a mildly reduced white cell count and low platelets.
  • The patient underwent two bone marrow biopsies, a percutaneous liver biopsy and had flow cytometry of her ascitic fluid, which revealed the diagnosis of a natural killer cell leukaemia.

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  • (PMID = 21886653.001).
  • [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/ PMC3031875
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74. Ketharanathan N, van Kipshagen PJ, Vasmel W, Barbé E, de Vries N: T/NK cell lymphoma presenting as a "blocked nose". Eur Arch Otorhinolaryngol; 2008 Sep;265(9):1131-4
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  • [Title] T/NK cell lymphoma presenting as a "blocked nose".
  • The diagnosis nasal T/NK cell lymphoma was established by histopathological investigation.
  • This case was diagnosed as stage IE lymphoma as no other sites were involved.
  • The nasal T/NK cell lymphoma is a rare malignancy, which is known to have an extremely aggressive and destructive course.
  • A high index of clinical suspicion is imperative to ensure early diagnosis and ultimately improve disease outcome.
  • [MeSH-major] Lymphoma, Extranodal NK-T-Cell / diagnosis. Nose Neoplasms / diagnosis
  • [MeSH-minor] Airway Obstruction / diagnosis. Airway Obstruction / etiology. Biomarkers, Tumor / analysis. Biopsy. Diagnosis, Differential. Fatal Outcome. Humans. Immunohistochemistry. Male. Middle Aged. Tomography, X-Ray Computed

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  • (PMID = 18188576.001).
  • [ISSN] 0937-4477
  • [Journal-full-title] European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
  • [ISO-abbreviation] Eur Arch Otorhinolaryngol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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75. Zheng YY, Chen G, Zhou XG, Jin Y, Xie JL, Zhang SH, Zhang YN: [Retrospective analysis of 4 cases of the so-called blastic NK-cell lymphoma, with reference to the 2008 WHO classification of tumours of haematopoietic and lymphoid tissues]. Zhonghua Bing Li Xue Za Zhi; 2010 Sep;39(9):600-5
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  • [Title] [Retrospective analysis of 4 cases of the so-called blastic NK-cell lymphoma, with reference to the 2008 WHO classification of tumours of haematopoietic and lymphoid tissues].
  • OBJECTIVE: To study the clinical and pathologic features of 4 cases of the so-called blastic natural killer (NK)-cell lymphoma, with reference to the 2008 WHO classification of tumours of haematopoietic and lymphoid tissues.
  • METHODS: The clinical, pathologic and immunohistochemical findings (EliVision method) of 4 cases of blastic NK-cell lymphoma (previously diagnosed according to the 2001 WHO classification) were retrospectively analyzed and reclassified with a special reference to the 2008 WHO classification.
  • RESULTS: The 4 cases of hematologic malignancy studied were characterized by the presence of medium-sized blastic lymphoma cells, CD56 expression, and absence of lineage-specific B-cell, T-cell and myeloid cell markers.
  • According to the 2001 WHO classification, they fell into the category of blastic NK-cell lymphoma.
  • They are likely derived from the plasmacytoid dendritic cells rather than NK cells.
  • They were then, according to the 2008 WHO classification, reclassified as the blastic plasmacytoid dendritic cell neoplasm.
  • This case was provisionally classified as a ambiguous lineage leukemia-NK cell lymphoblastic leukemia/lymphoma.
  • CONCLUSIONS: The so-called blastic NK-cell lymphomas in the 2001 WHO classification are rare and represent a heterogeneous group of lymphoproliferative disorders, with different clinical, pathologic and immunohistochemical features.
  • [MeSH-major] Precursor Cell Lymphoblastic Leukemia-Lymphoma / classification. Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology. Skin Neoplasms / classification. Skin Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Antigens, CD / metabolism. Antigens, CD56 / metabolism. Antigens, Differentiation, Myelomonocytic / metabolism. Humans. Interleukin-3 Receptor alpha Subunit / metabolism. Killer Cells, Natural / pathology. Middle Aged. Retrospective Studies. World Health Organization. Young Adult

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  • (PMID = 21092587.001).
  • [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] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD56; 0 / Antigens, Differentiation, Myelomonocytic; 0 / CD68 antigen, human; 0 / Interleukin-3 Receptor alpha Subunit
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76. Reimer P: Impact of autologous and allogeneic stem cell transplantation in peripheral T-cell lymphomas. Adv Hematol; 2010;2010:320624
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  • [Title] Impact of autologous and allogeneic stem cell transplantation in peripheral T-cell lymphomas.
  • Peripheral T/NK-cell lymphomas (PTCLs) are rare malignancies characterized by poor prognosis.
  • High-dose therapy and autologous stem cell transplantation (HDT-autoSCT) have shown good feasibility with low toxicity in retrospective studies.
  • In relapsing and refractory PTCL several comparison analyses suggest similar efficacy for PTCL when compared with aggressive B-cell lymphoma.
  • Allogeneic stem cell transplantation (alloSCT) has been investigated only as salvage treatment.
  • To define the role for upfront stem cell transplantation a randomised trial by the German High-Grade Non-Hodgkin Lymphoma Study Group comparing HDT-autoSCT and alloSCT will be initiated this year.

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  • (PMID = 21253465.001).
  • [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/ PMC3022174
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77. Ko YH, Park S, Kim K, Kim SJ, Kim WS: Aggressive natural killer cell leukemia: is Epstein-Barr virus negativity an indicator of a favorable prognosis? Acta Haematol; 2008;120(4):199-206
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  • [Title] Aggressive natural killer cell leukemia: is Epstein-Barr virus negativity an indicator of a favorable prognosis?
  • Aggressive natural killer (NK) cell leukemia (ANKL) is a prototype of an Epstein-Barr virus (EBV)-associated lymphoid malignancy, which is characterized by a fulminant clinical course and a median survival interval <2 months.
  • In conclusion, EBV-negative ANKL is an uncommon malignancy that pursues a less aggressive clinical course than EBV-positive ANKL.
  • [MeSH-major] Epstein-Barr Virus Infections / virology. Leukemia, T-Cell / virology
  • [MeSH-minor] Adolescent. Adult. Aged. Fatal Outcome. Female. Humans. Kaplan-Meier Estimate. Killer Cells, Natural / immunology. Male. Middle Aged. Precursor Cell Lymphoblastic Leukemia-Lymphoma / immunology. Recurrence. Young Adult

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  • [Copyright] Copyright 2009 S. Karger AG, Basel.
  • (PMID = 19153474.001).
  • [ISSN] 1421-9662
  • [Journal-full-title] Acta haematologica
  • [ISO-abbreviation] Acta Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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78. Sawada A, Sato E, Koyama M, Higuchi B, Kusuki S, Kim JY, Takeshita Y, Sakata A, Sakata N, Okamura T, Yasui M, Inoue M, Kawa K: NK-cell repertoire is feasible for diagnosing Epstein-Barr virus-infected NK-cell lymphoproliferative disease and evaluating the treatment effect. Am J Hematol; 2006 Aug;81(8):576-81
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  • [Title] NK-cell repertoire is feasible for diagnosing Epstein-Barr virus-infected NK-cell lymphoproliferative disease and evaluating the treatment effect.
  • Epstein-Barr virus (EBV) occasionally infects T and NK cells and causes EBV-infected T/NK-cell lymphoproliferative disease (LPD), which comprises chronic active EBV infection, EBV-associated hemophagocytic syndrome, mosquito allergy, hydroa vacciniforme, aggressive NK-cell leukemia, and NK/T-cell lymphoma.
  • The diagnosis is proven by the monoclonal proliferation of EBV-infected T or NK cells, which is a time-consuming and complicated method.
  • T-cell monoclonality is helpful for the screening of EBV-infected T-cell LPD in patients with EBV-genome burden and is easily shown with T-cell-receptor rearrangement or the T-cell repertoire, whereas NK-cell monoclonality is difficult to prove due to its lacking such rearranged receptors.
  • We investigated a set of killer immunoglobulin-like receptors (KIRs) and also CD94-NKG2 heterodimers on NK cells, namely the NK-cell repertoire.
  • Skewed repertoires were seen in all patients with EBV-infected NK-cell LPD, but not in any patients with EBV-infected T-cell LPD and were restored only after successful treatment.
  • The normal KIR repertoire is variable for each individual and it seems difficult to detect minimal residual EBV-infected lymphocytes.
  • However, the NK-cell repertoire is feasible for identifying EBV-infected NK-cell LPD and evaluating the treatment effect.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Epstein-Barr Virus Infections / immunology. Killer Cells, Natural / immunology. Killer Cells, Natural / virology. Lymphoproliferative Disorders / immunology. Stem Cell Transplantation

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  • (PMID = 16823820.001).
  • [ISSN] 0361-8609
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers
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79. Choi YL, Moriuchi R, Osawa M, Iwama A, Makishima H, Wada T, Kisanuki H, Kaneda R, Ota J, Koinuma K, Ishikawa M, Takada S, Yamashita Y, Oshimi K, Mano H: Retroviral expression screening of oncogenes in natural killer cell leukemia. Leuk Res; 2005 Aug;29(8):943-9
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  • [Title] Retroviral expression screening of oncogenes in natural killer cell leukemia.
  • Aggressive natural killer cell leukemia (ANKL) is an intractable malignancy that is characterized by the outgrowth of NK cells.
  • To identify transforming genes in ANKL, we constructed a retroviral cDNA expression library from an ANKL cell line KHYG-1.
  • Mutation-specific PCR analysis indicated that the KRAS mutation was present only in KHYG-1 cells, not in another ANKL cell line or in clinical specimens (n=8).
  • [MeSH-major] Gene Expression Regulation, Leukemic. Genetic Testing / methods. Killer Cells, Natural / metabolism. Leukemia / genetics. Oncogenes. Proto-Oncogene Proteins / genetics. Retroviridae / genetics
  • [MeSH-minor] 3T3 Cells. Animals. Cell Line. DNA, Complementary / genetics. Gene Library. Humans. Mice. Mutation. Transfection. ras Proteins

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  • (PMID = 15978945.001).
  • [ISSN] 0145-2126
  • [Journal-full-title] Leukemia research
  • [ISO-abbreviation] Leuk. Res.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / DNA, Complementary; 0 / KRAS protein, human; 0 / Proto-Oncogene Proteins; EC 3.6.5.2 / ras Proteins
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80. Ayyildiz O, Altintas A, Isikdogan A, Tuzcu A: Aggressive natural killer cell leukemia in a patient with common variable immunodeficiency syndrome. Gynecol Endocrinol; 2006 May;22(5):286-7
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  • [Title] Aggressive natural killer cell leukemia in a patient with common variable immunodeficiency syndrome.
  • [MeSH-major] Common Variable Immunodeficiency / complications. Killer Cells, Natural. Polyendocrinopathies, Autoimmune / complications. Precursor Cell Lymphoblastic Leukemia-Lymphoma / immunology

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  • [CommentOn] Gynecol Endocrinol. 2004 Jul;19(1):47-50 [15625773.001]
  • (PMID = 16785152.001).
  • [ISSN] 0951-3590
  • [Journal-full-title] Gynecological endocrinology : the official journal of the International Society of Gynecological Endocrinology
  • [ISO-abbreviation] Gynecol. Endocrinol.
  • [Language] eng
  • [Publication-type] Case Reports; Comment; Letter
  • [Publication-country] England
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81. Johansson AS, Norén-Nyström U, Larefalk A, Holmberg D, Lindskog M: Fish oil delays lymphoma progression in the TLL mouse. Leuk Lymphoma; 2010 Nov;51(11):2092-7
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  • [Title] Fish oil delays lymphoma progression in the TLL mouse.
  • The objective was to investigate the effects of omega-3 fatty acids, known for their anti-inflammatory effects, on time to lymphoma progression and survival in the TLL mouse, a strain genetically prone to developing aggressive T-cell lymphoma.
  • In contrast, omega-6 supplementation (corn oil) did not significantly affect lymphoma progression.
  • Immunological analysis demonstrated a significantly altered B-cell compartment and fewer NK cells in healthy C57Black6 mice fed omega-3, compared to controls.
  • In conclusion, a diet rich in omega-3 fatty acids delays lymphoma development in the TLL mouse possibly by mechanisms that include complex effects on immune function.
  • [MeSH-major] Fish Oils / pharmacology. Lymphoma, T-Cell / prevention & control
  • [MeSH-minor] Animals. B-Lymphocytes / drug effects. B-Lymphocytes / pathology. Corn Oil / pharmacology. Dietary Fats / pharmacology. Disease Models, Animal. Disease Progression. Fatty Acids, Omega-3 / pharmacology. Female. Killer Cells, Natural / drug effects. Killer Cells, Natural / pathology. Male. Mice. Mice, Inbred C57BL. Time Factors

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  • (PMID = 20919854.001).
  • [ISSN] 1029-2403
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Dietary Fats; 0 / Fatty Acids, Omega-3; 0 / Fish Oils; 8001-30-7 / Corn Oil
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82. Gubbels JA, Felder M, Horibata S, Belisle JA, Kapur A, Holden H, Petrie S, Migneault M, Rancourt C, Connor JP, Patankar MS: MUC16 provides immune protection by inhibiting synapse formation between NK and ovarian tumor cells. Mol Cancer; 2010 Jan 20;9:11
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  • [Title] MUC16 provides immune protection by inhibiting synapse formation between NK and ovarian tumor cells.
  • Here, we show that MUC16, a heavily glycosylated 3-5 million Da mucin expressed on the surface of ovarian tumor cells, inhibits the formation of immune synapses between NK cells and ovarian tumor targets.
  • RESULTS: Expression of low levels of MUC16 strongly correlated with an increased number of conjugates and activating immune synapses between ovarian tumor cells and primary naïve NK cells.
  • MUC16-knockdown ovarian tumor cells were more susceptible to lysis by primary NK cells than MUC16 expressing controls.
  • The NK cell leukemia cell line (NKL), which does not express KIRs but are positive for DNAM-1 and NKG2D, also conjugated and lysed MUC16-knockdown cells more efficiently than MUC16 expressing controls.
  • CONCLUSION: MUC16, a carrier of the tumor marker CA125, has previously been shown to facilitate ovarian tumor metastasis and inhibits NK cell mediated lysis of tumor targets.
  • Our data now demonstrates that MUC16 expressing ovarian cancer cells are protected from recognition by NK cells.

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  • (PMID = 20089172.001).
  • [ISSN] 1476-4598
  • [Journal-full-title] Molecular cancer
  • [ISO-abbreviation] Mol. Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA14520
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, Differentiation, T-Lymphocyte; 0 / CA-125 Antigen; 0 / CD226 antigen; 0 / Histocompatibility Antigens Class I; 0 / KLRK1 protein, human; 0 / Ligands; 0 / MUC16 protein, human; 0 / Membrane Proteins; 0 / NK Cell Lectin-Like Receptor Subfamily K
  • [Other-IDs] NLM/ PMC2818693
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83. Skorupa A, Chaudhary UB, Lazarchick J: Cold agglutinin induced autoimmune hemolytic anemia and NK-cell leukemia: a new association. Am J Hematol; 2007 Jul;82(7):668-71
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  • [Title] Cold agglutinin induced autoimmune hemolytic anemia and NK-cell leukemia: a new association.
  • Cold agglutinin induced autoimmune hemolytic anemia is uncommonly associated with leukemia and lymphomas.
  • We present a case of a young Mexican female presenting with a cold agglutinin hemolytic anemia with expression of a rare Pr antigen specificity and an aggressive NK-cell leukemia.
  • [MeSH-major] Anemia, Hemolytic, Autoimmune / metabolism. Anemia, Hemolytic, Autoimmune / pathology. Killer Cells, Natural / metabolism. Killer Cells, Natural / pathology. Leukemia / metabolism. Leukemia / pathology


84. Guerrero A M, Lira V P, Bertin C P, Galleguillos V M, Ocqueteau T M: [Natural killer cell leukemia. Case report]. Rev Med Chil; 2005 Apr;133(4):457-60
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Natural killer cell leukemia. Case report].
  • [Transliterated title] Leucemia de células natural killer. Caso clínico.
  • Natural killer leukemia is a rare and highly aggressive neoplasm, is more common in young male patients and has a very poor prognosis, with a median survival of few weeks.
  • Bone marrow aspiration and trephine biopsy showed an acute lymphoblastic leukemia.
  • The immunophenotype and immunohistochemistry revealed a natural killer acute leukemia.
  • The disease progressed rapidly and the patient died shortly after the diagnosis.
  • [MeSH-major] Killer Cells, Natural. Leukemia / pathology

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  • (PMID = 15953954.001).
  • [ISSN] 0034-9887
  • [Journal-full-title] Revista médica de Chile
  • [ISO-abbreviation] Rev Med Chil
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Chile
  • [Chemical-registry-number] 0 / Antigens, CD
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85. Grüllich C, Friske V, Finke J: Ex vivo detection of primary leukemia cells resistant to granule cytotoxin-induced cell death: a rapid isolation method to study granzyme-B-mediated cell death. Ann Hematol; 2008 Sep;87(9):701-8
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  • [Title] Ex vivo detection of primary leukemia cells resistant to granule cytotoxin-induced cell death: a rapid isolation method to study granzyme-B-mediated cell death.
  • Cytotoxic T lymphocytes and natural killer cells (CTL/NK) induce cell death in leukemia cells by the granzyme B (grB)-dependent granule cytotoxin (GC) pathway.
  • Resistance to GC may be involved in immune evasion of leukemia cells.
  • We developed a rapid method for the isolation of GC to investigate GC-mediated cell death in primary leukemia cells.
  • We isolated GC containing grB, grB complexes and PFN by detergent free hypotonic lysis of the human NK cell leukemia line YT.
  • The human leukemia cell lines KG-1, U937, K562 (myeloid leukemia), Jurkat, Daudi, and BV173 (lymphoblastic leukemia) treated with GC internalized grB and underwent cell death.
  • In primary leukemia cells analyzed ex vivo, we found GC-resistant leukemia cells in three out of seven patients with acute myeloid leukemia and one out of six patients with acute lymphoblastic leukemia.
  • We conclude that our method is fast (approximately 1 h) and yields active GC that induce grB-dependent cell death.
  • Furthermore, resistance to GC can be observed in acute leukemias and may be an important mechanism contributing to leukemia cell immune evasion.
  • [MeSH-major] Cell Death / drug effects. Cytotoxins / toxicity. Granzymes / toxicity. Leukemia / pathology
  • [MeSH-minor] Cell Line, Tumor. Drug Resistance, Neoplasm. Humans. K562 Cells / drug effects. K562 Cells / pathology. Killer Cells, Natural / immunology. Leukemia, Lymphocytic, Chronic, B-Cell / pathology. Leukemia, Myeloid, Acute / pathology. Perforin / metabolism. Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology. T-Lymphocytes, Cytotoxic / immunology. U937 Cells / drug effects. U937 Cells / pathology

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  • (PMID = 18437383.001).
  • [ISSN] 0939-5555
  • [Journal-full-title] Annals of hematology
  • [ISO-abbreviation] Ann. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Cytotoxins; 126465-35-8 / Perforin; EC 3.4.21.- / Granzymes
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86. Osuji N, Del Giudice I, Matutes E, Morilla A, Owusu-Ankomah K, Morilla R, Dunlop A, Catovksy D: CD52 expression in T-cell large granular lymphocyte leukemia--implications for treatment with alemtuzumab. Leuk Lymphoma; 2005 May;46(5):723-7
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  • [Title] CD52 expression in T-cell large granular lymphocyte leukemia--implications for treatment with alemtuzumab.
  • Few reports on the successful treatment of T-cell large granular lymphocyte (LGL) leukemia with the humanized anti-CD52 monoclonal antibody alemtuzumab are emerging in the literature.
  • Using semi-quantitative 2- and 3-color flow cytometry, we documented the expression of CD52 in 100% of abnormal cells in T-cell LGL leukemia (n = 11) and natural killer (NK) cell LGL leukemia (n = 2), and showed no significant difference in CD52 expression between T-cell prolymphocytic leukemia (PLL) and T-cell LGL leukemia.
  • Higher CD52 expression has been noted in responders to alemtuzumab in T-cell PLL and in chronic lymphocytic leukemia (CLL), a B-cell disorder.
  • The strong and consistent expression of CD52 shown here highlights the potential role of alemtuzumab in the treatment of refractory T-cell LGL leukemia and possibly aggressive NK cell leukemia.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antibodies, Neoplasm / therapeutic use. Antigens, CD / biosynthesis. Antigens, Neoplasm / biosynthesis. Antineoplastic Agents / therapeutic use. Glycoproteins / biosynthesis. Leukemia, Lymphoid / drug therapy. Leukemia, Lymphoid / immunology. Leukemia, T-Cell / drug therapy

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  • (PMID = 16019510.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antigens, CD; 0 / Antigens, Neoplasm; 0 / Antineoplastic Agents; 0 / CD52 antigen; 0 / Glycoproteins; 3A189DH42V / alemtuzumab
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87. Liang X, Graham DK: Natural killer cell neoplasms. Cancer; 2008 Apr 1;112(7):1425-36
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  • [Title] Natural killer cell neoplasms.
  • Natural killer (NK) cell tumors are an uncommon and heterogeneous group of disorders.
  • The World Health Organization (WHO) classified mature NK cell neoplasms into 2 types:.
  • 1) extranodal NK cell lymphoma, nasal type and 2) aggressive NK cell leukemia.
  • The mature NK cell tumors are prevalent in Asia and Central and South America.
  • Although blastic NK cell lymphoma, currently referred to as CD4+/CD56+ hematodermic neoplasm, also was included in the NK cell lymphoma category in the WHO classification scheme, existing evidence indicates a plasmacytoid dendritic cell derivation as opposed to an NK cell origin.
  • These tumors are characterized by blastic appearance, CD3-/CD4-/CD56+/CD13-/CD33- phenotype, T-cell receptor and immunoglobulin genes in germline configuration, and no evidence of EBV, suggesting a true immature NK cell derivation.
  • For this article, the authors reviewed the recent concepts and progress in clinicopathologic features, pathogenesis, genetic characteristics, diagnosis, differential diagnosis, treatment approaches, and outcomes of all subtypes of NK cell neoplasms.
  • [MeSH-major] Killer Cells, Natural / pathology. Leukemia / pathology. Lymphoma / pathology

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  • (PMID = 18286525.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 103
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