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1. Procházka V, Papajík T, Jarosová M, Pikalová Z, Indrák K, Tichý M, Kucerová L, Fakan F, Bílý M, Starostka D: T-cell gamma/delta hepatosplenic lymphoma - prolonged remission induced by aggressive first line treatment. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub; 2005 Dec;149(2):275-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] T-cell gamma/delta hepatosplenic lymphoma - prolonged remission induced by aggressive first line treatment.
  • T-cell gamma/delta hepatosplenic lymphoma is a primary extranodal lymphoma, distinct from other T/LGL lymphomas.
  • The clinical course is aggressive, and despite use of the multiagent chemotherapy and young age of the patients, the median survival is less than 1 year.
  • We reported a case of the successful intensive treatment with splenectomy, chemotherapy a first line autologous stem cell transplant.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Liver Neoplasms / drug therapy. Lymphoma, T-Cell, Peripheral / drug therapy. Receptors, Antigen, T-Cell, gamma-delta / analysis. Splenic Neoplasms / drug therapy

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  • (PMID = 16601770.001).
  • [ISSN] 1213-8118
  • [Journal-full-title] Biomedical papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
  • [ISO-abbreviation] Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Czech Republic
  • [Chemical-registry-number] 0 / Receptors, Antigen, T-Cell, gamma-delta
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2. Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE, Hosing C, Verstovsek S, Pro B: Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment. Ann Oncol; 2009 Jun;20(6):1080-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment.
  • BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare peripheral T-cell lymphoma; treatment with standard anthracycline-containing chemotherapy regimens has been disappointing, and an optimal treatment strategy for this patient population has not yet been determined.
  • Clinical characteristics and treatment results were reviewed.
  • RESULTS: Complete responses (CRs) were achieved in 7 of 14 patients who received chemotherapy.
  • Achievement of CR was followed by hematopoietic stem-cell transplantation in three patients.
  • Risk factors associated with worse outcome included male gender, failure to achieve a CR, history of immunocompromise, and absence of a T-cell receptor gene rearrangement in the gamma chain.
  • CONCLUSION: A better understanding of the pathophysiology of HSTCL and new therapeutic strategies are needed.

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  • (PMID = 19237479.001).
  • [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
  • [Other-IDs] NLM/ PMC4092251
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3. Tey SK, Marlton PV, Hawley CM, Norris D, Gill DS: Post-transplant hepatosplenic T-cell lymphoma successfully treated with HyperCVAD regimen. Am J Hematol; 2008 Apr;83(4):330-3
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  • [Title] Post-transplant hepatosplenic T-cell lymphoma successfully treated with HyperCVAD regimen.
  • Hepatosplenic T-cell lymphoma (HSTL) is an aggressive lymphoma.
  • We report successful treatment of post-transplant HSTL in a 50-year-old renal allograft recipient by reducing immunosuppression and using intensive chemotherapy consisting of alternating cycles of HyperCVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) and MTX/HiDAC (methotrexate, Ara-C).
  • High index of suspicion may lead to more timely diagnosis of this uncommon form of post-transplant lymphoproliferative disorder, and treatment with intensive chemotherapy such as HyperCVAD may be curative.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Liver Neoplasms / drug therapy. Lymphoma, T-Cell / drug therapy. Neoplasms, Second Primary / drug therapy. Postoperative Complications / drug therapy. Splenic Neoplasms / drug therapy
  • [MeSH-minor] Bone Marrow / pathology. Carcinoma, Transitional Cell / surgery. Cyclophosphamide / administration & dosage. Cytarabine / administration & dosage. Dexamethasone / administration & dosage. Doxorubicin / administration & dosage. Female. Humans. Immunosuppression / adverse effects. Kidney Transplantation. Methotrexate / administration & dosage. Middle Aged. Receptors, Antigen, T-Cell, gamma-delta / analysis. Remission Induction. Urinary Bladder Neoplasms / surgery. Vesico-Ureteral Reflux / surgery. Vincristine / administration & dosage

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17876769.001).
  • [ISSN] 1096-8652
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Receptors, Antigen, T-Cell, gamma-delta; 04079A1RDZ / Cytarabine; 5J49Q6B70F / Vincristine; 7S5I7G3JQL / Dexamethasone; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; YL5FZ2Y5U1 / Methotrexate; CVAD protocol
  • [Number-of-references] 24
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4. Gopcsa L, Bányai A, Tamáska J, Karádi A, Matolcsy A, Pálóczi K: Hepatosplenic gamma delta T-cell lymphoma with leukemic phase successfully treated with 2-chlorodeoxyadenosine. Haematologia (Budap); 2002;32(4):519-27
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hepatosplenic gamma delta T-cell lymphoma with leukemic phase successfully treated with 2-chlorodeoxyadenosine.
  • An unusual case of hepatosplenic gamma delta T-cell lymphoma with leukemic phase in a 39-year-old woman is reported.
  • Subsequently, she developed hepatomegaly and progressive neoplastic lymphocytosis.
  • Flowcytometric analysis of peripheral blood mononuclear cells demonstrated expression of CD3, CD7, CD16, CD56 antigens and T-cell receptor gamma delta.
  • A monoclonal TCR gamma- and beta-chain gene rearrangement were detected by polymerase chain reaction.
  • The patient was treated by traditional chemotherapy and alpha-interferon, unsuccessfully.
  • The reported case demonstrates the usefulness of 2-chlorodeoxyadenosine in treatment of hepatosplenic gamma delta T-cell lymphoma.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Cladribine / therapeutic use. Lymphoma, T-Cell / drug therapy. Lymphoma, T-Cell / immunology. Receptors, Antigen, T-Cell, gamma-delta / metabolism
  • [MeSH-minor] Adult. Female. Hepatomegaly / drug therapy. Humans. Recurrence. Remission Induction. Splenomegaly / drug therapy

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  • (PMID = 12803128.001).
  • [ISSN] 0017-6559
  • [Journal-full-title] Haematologia
  • [ISO-abbreviation] Haematologia (Budap)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Receptors, Antigen, T-Cell, gamma-delta; 47M74X9YT5 / Cladribine
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5. Grigg AP: 2'-Deoxycoformycin for hepatosplenic gammadelta T-cell lymphoma. Leuk Lymphoma; 2001 Aug;42(4):797-9
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  • [Title] 2'-Deoxycoformycin for hepatosplenic gammadelta T-cell lymphoma.
  • We describe a patient with unusually indolent hepatosplenic gammadelta T cell lymphoma characterised by severe lassitude and high fever.
  • This is the second recent report of gammadelta T cell lymphoma responding to dCF, suggesting this agent may have a specific role in this condition.
  • [MeSH-major] Antibiotics, Antineoplastic / administration & dosage. Liver Neoplasms / drug therapy. Lymphoma, T-Cell / drug therapy. Pentostatin / administration & dosage. Splenic Neoplasms / drug therapy
  • [MeSH-minor] Adult. Female. Humans. Receptors, Antigen, T-Cell, gamma-delta. Remission Induction

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  • (PMID = 11697510.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Receptors, Antigen, T-Cell, gamma-delta; 395575MZO7 / Pentostatin
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6. Gassas A, Kirby M, Weitzman S, Ngan B, Abla O, Doyle JJ: Hepatosplenic gammadelta T-cell lymphoma in a 10-year-old boy successfully treated with hematopoietic stem cell transplantation. Am J Hematol; 2004 Feb;75(2):113-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hepatosplenic gammadelta T-cell lymphoma in a 10-year-old boy successfully treated with hematopoietic stem cell transplantation.
  • The authors report a 10-year-old boy with hepatosplenic gammadelta T-cell lymphoma, a rare form of lymphoma that is highly aggressive, exceedingly rare in children, and primarily seen in young men.
  • Conventional multi-agent chemotherapy appears to be inadequate for cure.
  • This is the first report with this type of lymphoma in a boy less than 15 years old treated with hematopoietic stem cell transplantation (HSCT).
  • [MeSH-major] Liver Neoplasms / immunology. Lymphoma, T-Cell / immunology. Receptors, Antigen, T-Cell, gamma-delta / analysis. Splenic Neoplasms / immunology. Stem Cell Transplantation
  • [MeSH-minor] Child. Humans. Male. Treatment Outcome

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 14755380.001).
  • [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 / Receptors, Antigen, T-Cell, gamma-delta
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7. Weidmann E, Hinz T, Klein S, Schui DK, Harder S, Kriener S, Kabelitz D, Hoelzer D, Mitrou PS: Cytotoxic hepatosplenic gammadelta T-cell lymphoma following acute myeloid leukemia bearing two distinct gamma chains of the T-cell receptor. Biologic and clinical features. Haematologica; 2000 Oct;85(10):1024-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cytotoxic hepatosplenic gammadelta T-cell lymphoma following acute myeloid leukemia bearing two distinct gamma chains of the T-cell receptor. Biologic and clinical features.
  • BACKGROUND AND OBJECTIVES: Hepatosplenic gd T-cell lymphoma is a rare entity of peripheral T-cell lymphomas.
  • We characterized in detail the first case of hepatosplenic gd -T-cell lymphoma following acute myeloid leukemia.
  • DESIGN AND METHODS: Hepatosplenic gd -T-cell lymphoma was diagnosed in a woman who had been in complete remission (CR) of acute myeloid leukemia (AML) for two years.
  • Improvement but no objective response of the disease was observed after various types of chemotherapy.
  • CR was achieved after related donor stem cell transplantation.
  • Thirteen months later relapse of hepatosplenic gd T-cell lymphoma was diagnosed.
  • While being prepared for a second transplantation the patient developed meningeal lymphoma and died.
  • The patient's lymphoma cells were studied by immunologic, functional and molecular techniques.
  • RESULTS: Lymphoma cells expressed the gd T-cell receptor (TCR), CD2, CD3, CD5, CD7, CD38, CD45, CD161 (NKR-P1), TIA and Ki67.
  • Further analysis revealed expression of Vd1 and two distinct TCRg chains, Vg3 and Vg9, by the malignant cell clone.
  • Clone-specific PCR was negative at diagnosis of AML and was positive at all times during follow-up of the hepatosplenic gd T-cell lymphoma.
  • The lymphoma cells mediated strong natural killer cell-like cytotoxic activity, possibly explained by expression of CD161 and a lack of killer inhibitory receptor.
  • INTERPRETATION AND CONCLUSIONS: Several so far undescribed features were observed in this case of hepatosplenic gd T-cell lymphoma, such as T-cell lymphoma following AML, expression of two distinct T-cell receptor g-chains, and an unexpected cytotoxic phenotype.
  • [MeSH-major] Leukemia, Myeloid. Liver Neoplasms. Lymphoma, T-Cell. Neoplasms, Second Primary. Pregnancy Complications, Hematologic. Pregnancy Complications, Neoplastic. Splenic Neoplasms
  • [MeSH-minor] Acute Disease. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Female. Humans. Pregnancy. Receptors, Antigen, T-Cell, gamma-delta

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  • (PMID = 11025592.001).
  • [ISSN] 0390-6078
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] ITALY
  • [Chemical-registry-number] 0 / Receptors, Antigen, T-Cell, gamma-delta
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8. Corazzelli G, Capobianco G, Russo F, Frigeri F, Aldinucci D, Pinto A: Pentostatin (2'-deoxycoformycin) for the treatment of hepatosplenic gammadelta T-cell lymphomas. Haematologica; 2005 Mar;90(3):ECR14
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  • [Title] Pentostatin (2'-deoxycoformycin) for the treatment of hepatosplenic gammadelta T-cell lymphomas.
  • We report the results of treatment with single agent 2'-deoxycoformycin (Pentostatin) in two patients with Hepatosplenic gammadelta T-cell lymphoma (HSgammadeltaTCL), a rare lymphoma subtype with a highly unfavorable prognosis.
  • Further studies are warranted to define the optimal strategy to fully exploit therapeutic potential of this drug in patients with HSgammadeltaTCL.
  • [MeSH-major] Lymphoma, T-Cell / drug therapy. Pentostatin / therapeutic use. Receptors, Antigen, T-Cell, gamma-delta
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Humans. Liver Neoplasms / drug therapy. Splenic Neoplasms / drug therapy

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  • (PMID = 15753055.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Receptors, Antigen, T-Cell, gamma-delta; 395575MZO7 / Pentostatin
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9. Moleti ML, Testi AM, Giona F, Amendola A, Palumbo G, Uccini S, Starza ID, de Propris MS, Guarini A, Foà R: Gamma-delta hepatosplenic T-cell lymphoma. Description of a case with immunophenotypic and molecular follow-up successfully treated with chemotherapy alone. Leuk Lymphoma; 2006 Feb;47(2):333-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Gamma-delta hepatosplenic T-cell lymphoma. Description of a case with immunophenotypic and molecular follow-up successfully treated with chemotherapy alone.
  • This study hereby reports the case of a 19-year old boy with a gamma-delta hepatosplenic T-cell lymphoma (HSTCL).
  • Initial therapy consisted of four cycles of the IEV (Ifosphamide, Epirubicin and Etoposide) scheme.
  • Further treatment strategy was then adapted according to minimal residual disease monitoring by immunophenotypic and T-cell receptor gamma chain gene evaluation.
  • The patient remains in complete clinical, immunological and molecular remission and in good clinical conditions 48 months after diagnosis and 40 months after stopping therapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Lymphoma, T-Cell / drug therapy. Lymphoma, T-Cell / immunology. Receptors, Antigen, T-Cell, gamma-delta / biosynthesis
  • [MeSH-minor] Adult. Flow Cytometry. Follow-Up Studies. Hepatomegaly / etiology. Humans. Immunophenotyping. Male. Neoplasm, Residual. Remission Induction. Sensitivity and Specificity. Splenomegaly / etiology. Treatment Outcome

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  • (PMID = 16321867.001).
  • [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 / Receptors, Antigen, T-Cell, gamma-delta
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10. Domm JA, Thompson M, Kuttesch JF, Acra S, Frangoul H: Allogeneic bone marrow transplantation for chemotherapy-refractory hepatosplenic gammadelta T-cell lymphoma: case report and review of the literature. J Pediatr Hematol Oncol; 2005 Nov;27(11):607-10
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  • [Title] Allogeneic bone marrow transplantation for chemotherapy-refractory hepatosplenic gammadelta T-cell lymphoma: case report and review of the literature.
  • Hepatosplenic gammadelta T-cell lymphoma is an uncommon pediatric disease and is associated with an aggressive and often fatal course.
  • Liver biopsy and peripheral blood flow cytometry were diagnostic of hepatosplenic gammadelta T-cell lymphoma.
  • She was treated with multi-agent chemotherapy with cyclophosphamide, vincristine, prednisone, doxorubicin, and high-dose methotrexate but failed to achieve durable remission.
  • She underwent an allogeneic bone marrow transplant from her HLA-identical brother with a preparative regimen including total body irradiation and cyclophosphamide.
  • [MeSH-major] Bone Marrow Transplantation. Drug Resistance, Neoplasm. Liver Neoplasms / surgery. Lymphoma, T-Cell / surgery. Receptors, Antigen, T-Cell, gamma-delta / immunology. Splenic Neoplasms / surgery
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Combined Modality Therapy. Female. Flow Cytometry. Humans. Transplantation, Homologous. Whole-Body Irradiation

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  • (PMID = 16282893.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Receptors, Antigen, T-Cell, gamma-delta
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11. Mittal S, Milner BJ, Johnston PW, Culligan DJ: A case of hepatosplenic gamma-delta T-cell lymphoma with a transient response to fludarabine and alemtuzumab. Eur J Haematol; 2006 Jun;76(6):531-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of hepatosplenic gamma-delta T-cell lymphoma with a transient response to fludarabine and alemtuzumab.
  • Hepatosplenic gamma-delta T-cell lymphoma is a rare, usually fatal lymphoma and available literature on management is sparse.
  • We describe a further case with a dramatic, though transient response to Fludarabine and Alemtuzumab combination, following a failure of conventional chemotherapy.
  • Given the dreadful prognosis with conventional chemotherapy, it is a regimen worth pursuing as a disease reduction strategy prior to allograft where appropriate.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Liver / pathology. Lymphoma, T-Cell, Peripheral / drug therapy. Receptors, Antigen, T-Cell, gamma-delta / analysis. Spleen / pathology
  • [MeSH-minor] Adolescent. Antibodies, Monoclonal / administration & dosage. Antibodies, Monoclonal / adverse effects. Antibodies, Monoclonal, Humanized. Antibodies, Neoplasm / administration & dosage. Antibodies, Neoplasm / adverse effects. Antimetabolites, Antineoplastic / therapeutic use. Cisplatin. Combined Modality Therapy. Crohn Disease / complications. Cytarabine. Epirubicin / administration & dosage. Epstein-Barr Virus Infections / complications. Etoposide / administration & dosage. Fatal Outcome. Gene Rearrangement, delta-Chain T-Cell Antigen Receptor. Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor. Hematopoietic Stem Cell Transplantation. Humans. Ifosfamide / administration & dosage. Immunosuppressive Agents / adverse effects. Lymphoproliferative Disorders / etiology. Male. Methylprednisolone. Pentostatin / therapeutic use. Remission Induction. Transplantation, Homologous. Vidarabine / administration & dosage. Vidarabine / adverse effects. Vidarabine / analogs & derivatives

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  • (PMID = 16548918.001).
  • [ISSN] 0902-4441
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antimetabolites, Antineoplastic; 0 / Immunosuppressive Agents; 0 / Receptors, Antigen, T-Cell, gamma-delta; 04079A1RDZ / Cytarabine; 395575MZO7 / Pentostatin; 3A189DH42V / alemtuzumab; 3Z8479ZZ5X / Epirubicin; 6PLQ3CP4P3 / Etoposide; FA2DM6879K / Vidarabine; P2K93U8740 / fludarabine; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide; X4W7ZR7023 / Methylprednisolone; ESAP protocol; IEV protocol
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12. Gumbs AA, Zain J, Neylon E, MacGregor-Cortelli B, Patterson M, O'Connor OA: Importance of early splenectomy in patients with hepatosplenic T-cell lymphoma and severe thrombocytopenia. Ann Surg Oncol; 2009 Jul;16(7):2014-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Importance of early splenectomy in patients with hepatosplenic T-cell lymphoma and severe thrombocytopenia.
  • OBJECTIVE: T-cell lymphomas (TCLs) classically have a poorer response to therapy when compared with B-cell lymphomas and account for only 10-15% of all lymphoid malignancies.
  • Hepatosplenic TCLs are a rare subset of this group that usually present with hepatosplenomegaly, B-symptoms, and only rarely with lymphadenopathy.
  • BACKGROUND: This disease process, also known as gamma/delta (gamma/delta) TCL because of the expression of the T-cell receptor gamma/delta chain, tends to present in young male patients.
  • Hepatosplenic TCLs have recently gained notoriety because of the realization that patients with long-term treatment of some immunomodulators can develop this potentially fatal disease.
  • Unfortunately, patients with hepatosplenic TCL often present with thrombocytopenia and this new agent is contraindicated in these patients because of the potential exacerbation of thrombocytopenia with this agent.
  • RESULTS: Postoperatively the patient's thrombocytopenia resolved, permitting him to begin treatment with this potentially life-saving agent.
  • CONCLUSION: Due to the lethality of this disease and potential efficacy of new therapies, we believe splenectomy should be considered in patients with hepatosplenic lymphoma in an effort to improve the treatment options and survival of patients with this challenging disease.
  • [MeSH-major] Liver Neoplasms / surgery. Lymphoma, T-Cell / surgery. Splenectomy. Splenic Neoplasms / surgery. Thrombocytopenia / drug therapy
  • [MeSH-minor] Aminopterin / analogs & derivatives. Aminopterin / therapeutic use. Fatal Outcome. Hematologic Agents / therapeutic use. Humans. Laparoscopy. Male. Middle Aged. Treatment Outcome. Young Adult

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  • [ErratumIn] Ann Surg Oncol. 2009 Sep;16(9):2668. Neylon, Ellen [added]; MacGregor-Cortelli, Barbara [added]; Patterson, Molly [added]
  • (PMID = 19408055.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 10-propargyl-10-deazaaminopterin; 0 / Hematologic Agents; JYB41CTM2Q / Aminopterin
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13. Wu H, Wasik MA, Przybylski G, Finan J, Haynes B, Moore H, Leonard DG, Montone KT, Naji A, Nowell PC, Kamoun M, Tomaszewski JE, Salhany KE: Hepatosplenic gamma-delta T-cell lymphoma as a late-onset posttransplant lymphoproliferative disorder in renal transplant recipients. Am J Clin Pathol; 2000 Apr;113(4):487-96
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  • [Title] Hepatosplenic gamma-delta T-cell lymphoma as a late-onset posttransplant lymphoproliferative disorder in renal transplant recipients.
  • We report 2 cases of renal transplant recipients in whom hepatosplenic gamma-delta T-cell lymphoma (gamma-delta HSTCL) developed 5 and 10 years after transplantation.
  • The malignant cells showed typical immunophenotype of gamma-delta T cells (CD2+, CD3+, CD4-, CD8-, CD7+, gamma-delta T-cell receptor-positive, and alpha-beta T-cell receptor-negative) with clonal T-cell receptor gene rearrangement and were of the V-delta-1 subset.
  • In addition, the cells contained a cytolytic granule-associated protein, TIA-1, and Fas ligand, indicating cytotoxic T-cell differentiation.
  • The malignant T cells in both cases were of host tissue origin.
  • Despite initial response to chemotherapy, both patients died within 6 months of diagnosis.
  • Our findings indicate that gamma-delta HSTCL can occur as a late complication in transplant recipients.
  • [MeSH-major] Kidney Transplantation / adverse effects. Liver Neoplasms / etiology. Lymphoma, T-Cell / etiology. Proteins. Receptors, Antigen, T-Cell, gamma-delta / immunology. Splenic Neoplasms / etiology
  • [MeSH-minor] Adult. DNA, Neoplasm / analysis. Fas Ligand Protein. Fatal Outcome. Female. Gene Rearrangement, delta-Chain T-Cell Antigen Receptor / genetics. Humans. Immunophenotyping. In Situ Hybridization, Fluorescence. Male. Membrane Glycoproteins / metabolism. Membrane Proteins / metabolism. Middle Aged. Poly(A)-Binding Proteins. Polymerase Chain Reaction. RNA-Binding Proteins / metabolism. Receptors, Interleukin-2 / blood

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  • (PMID = 10761449.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / FASLG protein, human; 0 / Fas Ligand Protein; 0 / Membrane Glycoproteins; 0 / Membrane Proteins; 0 / Poly(A)-Binding Proteins; 0 / Proteins; 0 / RNA-Binding Proteins; 0 / Receptors, Antigen, T-Cell, gamma-delta; 0 / Receptors, Interleukin-2; 0 / TIA1 protein, human
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14. Chin M, Mugishima H, Takamura M, Nagata T, Shichino H, Shimada T, Suzuki T, Harada K, Imashuku S, Yokota S: Hemophagocytic syndrome and hepatosplenic gammadelta T-cell lymphoma with isochromosome 7q and 8 trisomy. J Pediatr Hematol Oncol; 2004 Jun;26(6):375-8
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  • [Title] Hemophagocytic syndrome and hepatosplenic gammadelta T-cell lymphoma with isochromosome 7q and 8 trisomy.
  • The authors describe a 15-year-old boy with hepatosplenic gammadelta T-cell lymphoma associated with hemophagocytic syndrome (HPS) along with isochromosome 7q and trisomy 8.
  • Physical examination, radiography, and ultrasound tomography revealed no lymphoadenopathy.
  • He had elevated levels of serum ferritin, interferon-gamma, soluble interleukin-2 receptor, and interleukin-6.
  • Clonal rearrangement of the genes for T-cell receptor gamma and delta chains was elucidated by polymerase chain reaction.
  • He achieved a complete remission after intensive chemotherapy and underwent splenectomy 18 months after diagnosis.
  • This might mean that this type of lymphoma is refractory, as reported previously, and might indicate that marrow ablative therapy is needed to achieve a cure.
  • The present case illustrates the usefulness of MRD analysis, and MRD studies in this group of disorders may be helpful in the decision of whether to continue a more aggressive therapeutic approach.
  • [MeSH-major] Chromosomes, Human, Pair 7 / genetics. Chromosomes, Human, Pair 8 / genetics. Histiocytosis / genetics. Lymphoma, T-Cell / genetics. Receptors, Antigen, T-Cell, gamma-delta / genetics. Trisomy / genetics
  • [MeSH-minor] Base Sequence. DNA Primers. Gene Rearrangement, delta-Chain T-Cell Antigen Receptor / genetics. Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / genetics. Humans. Isochromosomes / genetics. Male. Middle Aged. Polymerase Chain Reaction

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  • (PMID = 15167351.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA Primers; 0 / Receptors, Antigen, T-Cell, gamma-delta
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15. Dueck GS, Chua N, Prasad A, Stewart D, White D, vanderJagt R, Johnston JB, Belch A, Reiman T: Activity of lenalidomide in a phase II trial for T-cell lymphoma: Report on the first 24 cases. J Clin Oncol; 2009 May 20;27(15_suppl):8524

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Activity of lenalidomide in a phase II trial for T-cell lymphoma: Report on the first 24 cases.
  • : 8524 Background: Novel therapies are needed to improve outcomes in T-cell lymphomas.
  • We report the interim results of a prospective multicenter trial evaluating lenalidomide in T-cell lymphomas.
  • METHODS: Patients with relapsed and refractory T-cell lymphomas other than mycosis fungoides were prescribed oral lenalidomide (25mg daily) on days 1 to 21 of each 28 day cycle, with standardized dose reductions for toxicity.
  • Treatment continued until disease progression, death or unacceptable toxicity.
  • RESULTS: At the time of this interim analysis, 24 patients were enrolled in this study and 23 were evaluable for response.
  • The histology was peripheral T-cell unspecified (PTCL-u, n=10), angioimmunoblastic (n=7), anaplastic large cell (n=5), enteropathic T-cell (n=1) and hepatosplenic gamma/delta (n=1).
  • Median number of prior therapies was 1 (range, 0-4), and three had prior autologous stem cell transplant.
  • Four patients were previously untreated and not candidates for combination chemotherapy.
  • Median time from completion of prior therapy to the start of lenalidomide was 8 months (range, 1-48 months).
  • The most common grade 3 adverse events were neutropenia (20.8%), febrile neutropenia (16.7%), and pain NOS (16.7%).
  • CONCLUSIONS: In relapsed and refractory T-cell lymphomas, oral lenalidomide monotherapy has clinical activity and toxicity is consistent with the known profile of lenalidomide.

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  • (PMID = 27960899.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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16. Motta G, Vianello F, Menin C, De Nicolo A, Agata S, Altavilla G, Pietrogrande F, Girolami A: Hepatosplenic gammadelta T-cell lymphoma presenting with immune-mediated thrombocytopenia and hemolytic anemia (Evans' syndrome). Am J Hematol; 2002 Apr;69(4):272-6
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  • [Title] Hepatosplenic gammadelta T-cell lymphoma presenting with immune-mediated thrombocytopenia and hemolytic anemia (Evans' syndrome).
  • We describe an unusual case of hepatosplenic T-cell lymphoma in a 61-year-old man who presented with fever, hepatosplenomegaly, anemia, and thrombocytopenia.
  • A spleen biopsy was consistent with T-cell lymphoma.
  • Using the polymerase chain reaction approach, clonality of the T-cell receptor gamma-chain gene rearrangement could be demonstrated, while Southern blot analysis disclosed only a germline configuration of the T-cell receptor beta chain genes.
  • Of interest, an immune-mediated mechanism was demonstrated and was most likely responsible for erythrocyte and platelet destruction; this is, therefore, the first report of gamma T-cell lymphoma in association with Evans' syndrome.
  • Initial steroid treatment was efficacious in limiting autoimmunity but constitutional symptoms did not subside.
  • Chemotherapy (MACOP-B) was successful in obtaining complete clinical remission.
  • Finally, thrombocytopenia in gammadelta T-cell lymphoma patients should be routinely evaluated for platelet autoantibodies.
  • [MeSH-major] Anemia, Hemolytic / immunology. Autoimmune Diseases / complications. Liver Neoplasms / diagnosis. Lymphoma, T-Cell / diagnosis. Splenic Neoplasms / diagnosis. Thrombocytopenia / immunology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Autoantibodies / blood. Biopsy. Bleomycin / therapeutic use. Blood Platelets / immunology. Bone Marrow / pathology. Cyclophosphamide / therapeutic use. Doxorubicin / therapeutic use. Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor. Hepatomegaly. Humans. Hydrocortisone / therapeutic use. Leucovorin / therapeutic use. Male. Methotrexate / therapeutic use. Middle Aged. Prednisone / therapeutic use. Receptors, Antigen, T-Cell, gamma-delta / analysis. Remission Induction. Splenomegaly. Syndrome. Vincristine / therapeutic use

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  • [Copyright] Copyright 2002 Wiley-Liss, Inc.
  • (PMID = 11921021.001).
  • [ISSN] 0361-8609
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Autoantibodies; 0 / Receptors, Antigen, T-Cell, gamma-delta; 11056-06-7 / Bleomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; Q573I9DVLP / Leucovorin; VB0R961HZT / Prednisone; WI4X0X7BPJ / Hydrocortisone; YL5FZ2Y5U1 / Methotrexate; MACOP-B protocol
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17. Macon WR, Levy NB, Kurtin PJ, Salhany KE, Elkhalifa MY, Casey TT, Craig FE, Vnencak-Jones CL, Gulley ML, Park JP, Cousar JB: Hepatosplenic alphabeta T-cell lymphomas: a report of 14 cases and comparison with hepatosplenic gammadelta T-cell lymphomas. Am J Surg Pathol; 2001 Mar;25(3):285-96
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  • [Title] Hepatosplenic alphabeta T-cell lymphomas: a report of 14 cases and comparison with hepatosplenic gammadelta T-cell lymphomas.
  • Hepatosplenic gammadelta T-cell lymphoma is a distinct entity, characterized by occurrence in young adult males with hepatosplenomegaly, B-symptoms, peripheral blood cytopenias, and no lymphadenopathy; lymphomatous infiltrates in the splenic red pulp, hepatic sinusoids, and bone marrow sinuses; T-cell receptor (TCR) gammadelta chains and a cytotoxic T-cell phenotype; isochromosome 7q; and an aggressive clinical course.
  • In comparison, this study describes the clinicopathologic features of 14 hepatosplenic T-cell lymphomas expressing TCR alphabeta chains.
  • They occurred in 11 women and 3 men with a median age of 36 years.
  • Clinical presentation was similar to that described previously for hepatosplenic gammadelta T-cell lymphomas, except for the female preponderance and age distribution (5 patients younger than 13 years of age and 5 patients older than 50 years of age).
  • Tumor cells in all 14 lymphomas were cytotoxic alphabeta T-cells; 13 co-expressed natural killer cell-associated antigens and showed T-cell clonality.
  • Eleven patients are dead, eight within a year of diagnosis, and two patients have maintained complete remissions after combination chemotherapy.
  • These data show that hepatosplenic T-cell lymphomas include an alphabeta-subtype.
  • [MeSH-major] Liver Neoplasms / pathology. Lymphoma, T-Cell / pathology. Receptors, Antigen, T-Cell, alpha-beta / metabolism. Receptors, Antigen, T-Cell, gamma-delta / metabolism. Splenic Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Bone Marrow / pathology. Child. Child, Preschool. DNA, Neoplasm / analysis. Female. Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / genetics. Humans. Infant. Infant, Newborn. Karyotyping. Lymph Nodes / pathology. Male. Middle Aged. Polymerase Chain Reaction

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  • (PMID = 11224598.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Receptors, Antigen, T-Cell, alpha-beta; 0 / Receptors, Antigen, T-Cell, gamma-delta
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18. Niitsu N, Kohri M, Togano T, Nakamine H, Nakamura S, Iwabuchi K, Higashihara M: Development of hepatosplenic gammadelta T-cell lymphoma with pancytopenia during early pregnancy: a case report and review of the literature. Eur J Haematol; 2004 Nov;73(5):367-71
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  • [Title] Development of hepatosplenic gammadelta T-cell lymphoma with pancytopenia during early pregnancy: a case report and review of the literature.
  • Lymphomas rarely develop during pregnancy, but hepatosplenic gammadelta T-cell lymphoma (HSgammadeltaTCL) is extremely rare.
  • We encountered a case of T-cell intracellular antigen-1 (TIA-1) positive and granzyme B-positive HSgammadeltaTCL that developed early during the course of pregnancy.
  • The patient was a 31-yr-old female who was referred to our hospital because of pancytopenia and splenomegaly at the time of the14th week of her gestation.
  • They were CD3epsilon+, mCD3+, CD4-, CD8-, CD56+, CD79a-, T-cell receptor (TCR)-gammadelta protein+, TIA-1+, and granzyme B+ by either immunohistochemistry or flow cytometry.
  • Clonal rearrangement of TCR-gamma genes without such rearrangement of TCR-delta and TCR-beta genes was confirmed by Southern blot hybridization.
  • Thus, the patient was diagnosed as having HSgammadeltaTCL, and combination chemotherapy was initiated.
  • To our knowledge, this is the first case report of HSgammadeltaTCL that developed during pregnancy.
  • Pathogenesis of pregnancy-associated lymphoma is not known, but it is possible that maternal immunity during pregnancy or a hormonal imbalance, such as a change in the progesterone level, induces the development of lymphoma.
  • Pregnancy-associated lymphoma is resistant to standard chemotherapy and is associated with poor prognosis.
  • Therefore, it is important to accumulate clinicopathologic data of such cases for the development of a treatment modality.
  • [MeSH-major] Liver Neoplasms / diagnosis. Lymphoma, T-Cell / diagnosis. Pancytopenia. Pregnancy Complications, Neoplastic. Receptors, Antigen, T-Cell, gamma-delta / analysis. Splenic Neoplasms / diagnosis
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cesarean Section. Female. Gestational Age. Humans. Immunohistochemistry. Immunophenotyping. Pregnancy. Remission Induction. Spleen / chemistry. Spleen / pathology. Spleen / surgery. Splenectomy

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  • (PMID = 15458516.001).
  • [ISSN] 0902-4441
  • [Journal-full-title] European journal of haematology
  • [ISO-abbreviation] Eur. J. Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Receptors, Antigen, T-Cell, gamma-delta
  • [Number-of-references] 14
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19. Harada Y, Kato S, Komiya H, Shirota T, Mukai K, Hayashi T: Primary omental gamma/delta T-cell lymphoma involving the central nervous system. Leuk Lymphoma; 2004 Sep;45(9):1947-50
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  • [Title] Primary omental gamma/delta T-cell lymphoma involving the central nervous system.
  • Gamma/delta T-cell lymphoma (GDTL) is an uncommon lymphoma that was initially reported to involve only the liver and spleen.
  • GDTL other than the hepatosplenic type is extremely rare.
  • Frequent primary sites include skin and subcutaneous tissue, intestine, or nasal region.
  • During the fourth course of EPOCH, muscle weakness of the lower extremities developed and intracranial masses were observed by computed tomographic scan of the brain.
  • Dissemination of lymphoma to the central nervous system was considered and it may be attributable to the expression of CD56 in this case.
  • High dose methotrexate (HD-MTX) chemotherapy successfully eliminated the omental tumor and reduced the size of the intracranial masses, thus HD-MTX appears to be an effective treatment against GDTL.
  • [MeSH-major] Central Nervous System / pathology. Lymphoma, T-Cell / pathology. Omentum / pathology. Peritoneal Neoplasms / pathology
  • [MeSH-minor] Humans. Japan. Male. Middle Aged. Tomography, X-Ray Computed

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  • [Copyright] Copyright 2004 Taylor and Francis Ltd
  • (PMID = 15223661.001).
  • [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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20. Navarro JT, Ribera JM, Mate JL, Granada I, Juncà J, Batlle M, Millá F, Feliu E: Hepatosplenic T-gammadelta lymphoma in a patient with Crohn's disease treated with azathioprine. Leuk Lymphoma; 2003 Mar;44(3):531-3
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  • [Title] Hepatosplenic T-gammadelta lymphoma in a patient with Crohn's disease treated with azathioprine.
  • Hepatosplenic gammadelta T-cell lymphoma (HS-gammadeltaTCL) is an uncommon type of peripheral T-cell lymphoma, which has been associated in some cases with immunosuppression, mainly after solid organ transplants.
  • We describe a case of HSgammadeltaTCL with a leukaemic course in a patient with Crohn's disease who had received azathioprine during the previous 5.5 years.
  • The patient did not respond to intensive chemotherapy.
  • [MeSH-major] Autoimmune Diseases / complications. Crohn Disease / complications. Glucosamine / analogs & derivatives. Liver Neoplasms / complications. Lymphoma, T-Cell, Peripheral / complications. Receptors, Antigen, T-Cell, gamma-delta / analysis. Splenic Neoplasms / complications
  • [MeSH-minor] Adrenal Cortex Hormones / therapeutic use. Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Azathioprine / adverse effects. Azathioprine / therapeutic use. Chromosome Aberrations. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Doxorubicin / administration & dosage. Drug Combinations. Fatal Outcome. Humans. Immunophenotyping. Immunosuppressive Agents / adverse effects. Immunosuppressive Agents / therapeutic use. Male. Prednisone / administration & dosage. Splenectomy. Sulfasalazine / therapeutic use. Vincristine / administration & dosage

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  • (PMID = 12688327.001).
  • [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 / Adrenal Cortex Hormones; 0 / Drug Combinations; 0 / Immunosuppressive Agents; 0 / Receptors, Antigen, T-Cell, gamma-delta; 31363-03-8 / Salazopyrine; 3XC8GUZ6CB / Sulfasalazine; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; MRK240IY2L / Azathioprine; N08U5BOQ1K / Glucosamine; VB0R961HZT / Prednisone; CHOP protocol
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21. Taguchi A, Miyazaki M, Sakuragi S, Shinohara K, Kamei T, Inoue Y: Gamma/delta T cell lymphoma. Intern Med; 2004 Feb;43(2):120-5
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  • [Title] Gamma/delta T cell lymphoma.
  • The pathological findings of a liver biopsy specimen revealed the infiltration of lymphocytes in the sinusoids and that of the laparoscopically resected spleen revealed the infiltration of lymphocytes in the red pulp, which was positive for CD3, CD43, CD45RO and T-cell intracellular antigen-1 (TIA-1) and was negative for betaF1, while the white pulp was spared.
  • Genetic analysis of the spleen cells revealed the rearrangement of T-cell receptor (TCR) Cbeta1, Jdelta1 and Jgamma.
  • Hepatosplenic gammadelta T cell lymphoma (GDTL) was diagnosed.
  • The patient was treated with chemotherapy by cyclophosphamide, hydroxydoxorubicin, vincristine and prednisolone (CHOP), however, it was ineffective, and the patient died of hemorrhage from the lymphoma involvement of the intestine 5 months after the onset of disease.
  • [MeSH-major] Liver Neoplasms / genetics. Lymphoma, T-Cell, Peripheral / genetics. Receptors, Antigen, T-Cell, gamma-delta / genetics. Splenic Neoplasms / genetics
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow Cells / physiology. Cyclophosphamide / therapeutic use. DNA, Viral / analysis. Doxorubicin / therapeutic use. Female. Flow Cytometry. Gene Rearrangement, T-Lymphocyte. Hepatomegaly. Herpesvirus 4, Human / genetics. Humans. Immunohistochemistry. Liver / metabolism. Liver / pathology. Middle Aged. Prednisolone / therapeutic use. Spleen / metabolism. Spleen / pathology. Splenomegaly. Vascular Endothelial Growth Factor A / blood. Vincristine / therapeutic use

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  • [CommentIn] Intern Med. 2004 Feb;43(2):83-4 [15005247.001]
  • (PMID = 15005254.001).
  • [ISSN] 0918-2918
  • [Journal-full-title] Internal medicine (Tokyo, Japan)
  • [ISO-abbreviation] Intern. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / DNA, Viral; 0 / Receptors, Antigen, T-Cell, gamma-delta; 0 / Vascular Endothelial Growth Factor A; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; 9PHQ9Y1OLM / Prednisolone; VAP-cyclo protocol
  • [Number-of-references] 19
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22. Iyama S, Matsunaga T, Sato T, Sato Y, Sagawa T, Murase K, Kida M, Kobune M, Takimoto R, Kato J, Niitsu Y: [A case of hepatosplenic gammadelta T-cell lymphoma associated hemophagocytic syndrome]. Gan To Kagaku Ryoho; 2008 Sep;35(9):1623-7
MedlinePlus Health Information. consumer health - Liver Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [A case of hepatosplenic gammadelta T-cell lymphoma associated hemophagocytic syndrome].
  • A 56-year-old woman was admitted to our hospital for examination of high fever, liver dysfunction, pancytopenia, elevated lactate dehydrogenase (LDH) and ferritin, which were not improved by methylprednisolone pulse therapy.
  • She was then treated with chemotherapy consisting of etoposide, prednisolone and cyclosporine, which resulted in transient improvement and allowed her to undergo whole-body fluorine-18fluorodeoxyglucose positron emission tomography (FDG-PET) analysis.
  • A liver biopsy was performed and the examination of the specimen with immunohistochemical staining and PCR analysis revealed monoclonal infiltration of gammadelta T-cell.
  • Despite the repeated chemotherapy, she deteriorated rapidly and succumbed to multi-organ failure.
  • [MeSH-major] Liver Neoplasms / metabolism. Liver Neoplasms / pathology. Lymphohistiocytosis, Hemophagocytic / pathology. Lymphoma, T-Cell / pathology. Receptors, Antigen, T-Cell, gamma-delta / metabolism. Splenic Neoplasms / metabolism. Splenic Neoplasms / pathology
  • [MeSH-minor] Biopsy. Female. Humans. Middle Aged. Positron-Emission Tomography. Tomography, X-Ray Computed

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  • (PMID = 18799926.001).
  • [ISSN] 0385-0684
  • [Journal-full-title] Gan to kagaku ryoho. Cancer & chemotherapy
  • [ISO-abbreviation] Gan To Kagaku Ryoho
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Receptors, Antigen, T-Cell, gamma-delta
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23. Tripodo C, Iannitto E, Florena AM, Pucillo CE, Piccaluga PP, Franco V, Pileri SA: Gamma-delta T-cell lymphomas. Nat Rev Clin Oncol; 2009 Dec;6(12):707-17
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  • [Title] Gamma-delta T-cell lymphomas.
  • Peripheral T-cell lymphomas (TCLs) are uncommon neoplasms, accounting for about 12% of all lymphoid tumors worldwide.
  • TCLs in which gammadelta T-cell receptors are expressed (gammadelta TCLs) are extremely aggressive and rare (<1% of lymphoid neoplasms).
  • Two distinct gammadelta TCL entities are recognized: hepatosplenic T-cell lymphoma (HSTL) and primary cutaneous gammadelta T-cell lymphoma (PCGD-TCL).
  • HSTL is a well-characterized extranodal lymphoma that has a disguised onset, secondary to intrasinusoidal infiltration of the spleen, liver and bone marrow, has a rapidly progressive course that is poorly responsive to chemotherapy, and often ensues in the setting of immune system suppression.
  • PCGD-TCL can present with prominent epidermal involvement or with a panniculitis-like clinical picture that can be complicated by a concurrent hemophagocytic syndrome; the disease shows biological and phenotypic overlap with other extranodal gammadelta TCLs that involve the respiratory or gastrointestinal tract mucosa.
  • In this Review, we discuss the clinical and biological features, the diagnostic challenges and the therapeutic perspectives of HSTL and PCGD-TCL.
  • [MeSH-major] Lymphoma, T-Cell, Cutaneous / diagnosis. Lymphoma, T-Cell, Peripheral / diagnosis. Receptors, Antigen, T-Cell, gamma-delta / genetics
  • [MeSH-minor] Clinical Trials as Topic. Gene Rearrangement, delta-Chain T-Cell Antigen Receptor / genetics. Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / genetics. Humans

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  • (PMID = 19901919.001).
  • [ISSN] 1759-4782
  • [Journal-full-title] Nature reviews. Clinical oncology
  • [ISO-abbreviation] Nat Rev Clin Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Receptors, Antigen, T-Cell, gamma-delta
  • [Number-of-references] 87
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24. Jiang L, Abati AD, Wilson W, Stetler-Stevenson M, Yuan C: Persistent non-neoplastic gammadelta-T cells in cerebrospinal fluid of a patient with hepatosplenic (gammadelta) T cell lymphoma: a case report with 6 years of flow cytometry follow-up. Int J Clin Exp Pathol; 2009;3(1):110-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Persistent non-neoplastic gammadelta-T cells in cerebrospinal fluid of a patient with hepatosplenic (gammadelta) T cell lymphoma: a case report with 6 years of flow cytometry follow-up.
  • Hepatosplenic (gammadelta) T-cell lymphoma (HSTCL) is an uncommon T-cell lymphoma with an aggressive clinical course and poor prognosis.
  • We describe a case of a 31-year old man diagnosed with a gammadelta HSTCL in 2003, successfully treated with chemotherapy and allogeneic stem cell transplantation, and followed from 2003 to present.
  • Molecular studies for T-cell gene rearrangement were non-contributory.
  • Comparison to in-house cases from patients with hairy cell leukemia and concomitant increases in non-neoplastic gammadelta T-cells was performed.
  • We describe an unusual case of persistent gammadelta T-cells in the CSF of a patient during 6 years of flow cytometric follow-up after treatment for gammadelta HSTCL.
  • FC analysis helps to make this distinction, even with a limited panel.
  • [MeSH-major] Flow Cytometry / methods. Liver Neoplasms / pathology. Lymphoma, T-Cell / pathology. Receptors, Antigen, T-Cell, gamma-delta / analysis. Splenic Neoplasms / pathology. T-Lymphocytes / pathology
  • [MeSH-minor] Adult. Biopsy. Combined Modality Therapy. Disease-Free Survival. Follow-Up Studies. Humans. Immunophenotyping. Male. Stem Cell Transplantation

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  • (PMID = 19918335.001).
  • [ISSN] 1936-2625
  • [Journal-full-title] International journal of clinical and experimental pathology
  • [ISO-abbreviation] Int J Clin Exp Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Receptors, Antigen, T-Cell, gamma-delta
  • [Other-IDs] NLM/ PMC2776263
  • [Keywords] NOTNLM ; Gamma-delta T cells / T-cell gene rearrangement / cerebrospinal fluid / flow cytometry / hepatosplenic T cell lymphoma
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25. Aldinucci D, Poletto D, Zagonel V, Rupolo M, Degan M, Nanni P, Gattei V, Pinto A: In vitro and in vivo effects of 2'-deoxycoformycin (Pentostatin) on tumour cells from human gammadelta+ T-cell malignancies. Br J Haematol; 2000 Jul;110(1):188-96
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  • [Title] In vitro and in vivo effects of 2'-deoxycoformycin (Pentostatin) on tumour cells from human gammadelta+ T-cell malignancies.
  • Hepatosplenic gammadelta+ T-cell lymphoma represents a rare neoplasm of post-thymic phenotype, characterized by an aggressive clinical course and a poor response to conventional chemotherapy.
  • In the present study, we have examined the cytotoxic effects of the purine analogue 2'-deoxycoformycin (dCF) on cultured mononuclear cells and purified gammadelta+ tumour cells from bone marrow or peripheral blood of four patients with hepatosplenic gammadelta+ T-cell lymphoma.
  • After 48 h of in vitro exposure to dCF, the absolute number of viable CD3+/gammadelta+ tumour T cells was reduced by more than 90% in all samples with respect to control cultures, with absolute counts of viable CD3+/alphabeta+ lymphocytes being reduced only by 6-40% of the initial cell input.
  • Analysis of cultures after 5 d of exposure to dCF plus dAdo revealed the persistence of normal CD3+/alphabeta+ T cells, which accounted, however, for only 20-25% of the initial cell input.
  • We also report that one patient with hepatosplenic gammadelta+ T-cell lymphoma in terminal leukaemic phase showed a striking haematological response to single-agent dCF given as fourth-line treatment.
  • In particular, the selective clearance of gammadelta+ tumour T cells in peripheral blood and bone marrow was observed starting after the second course of treatment.
  • Our results suggest that dCF may represent a potentially active drug for the management of this aggressive form of T-cell lymphoma.
  • [MeSH-major] Adenosine Deaminase Inhibitors. Liver Neoplasms / drug therapy. Lymphoma, T-Cell / drug therapy. Pentostatin / pharmacology. Receptors, Antigen, T-Cell, gamma-delta. Splenic Neoplasms / drug therapy
  • [MeSH-minor] Adult. Enzyme Inhibitors / pharmacology. Female. Flow Cytometry. Humans. Immunophenotyping. Male. Middle Aged. Tumor Cells, Cultured / drug effects

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  • (PMID = 10930997.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] 0 / Adenosine Deaminase Inhibitors; 0 / Enzyme Inhibitors; 0 / Receptors, Antigen, T-Cell, gamma-delta; 395575MZO7 / Pentostatin
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26. Meijer JW, Mulder CJ, Goerres MG, Boot H, Schweizer JJ: Coeliac disease and (extra)intestinal T-cell lymphomas: definition, diagnosis and treatment. Scand J Gastroenterol Suppl; 2004;(241):78-84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Coeliac disease and (extra)intestinal T-cell lymphomas: definition, diagnosis and treatment.
  • Intestinal lymphomas encompass those lymphomas with a dominant or only localized occurrence in the intestinal tract.
  • Coeliac disease is highly associated with enteropathy-associated T-cell lymphomas (EATLs).
  • EATL is often multifocal with ulcerative lesions, which explains the high perforation rate at presentation or during chemotherapy.
  • Positron emission tomography (PET) scanning may be valuable.
  • There is no satisfactory treatment for EATL, the only possibility of preventing EATL development in RCD being autologous bone marrow transplantation.
  • EATLs can present in 20% of patients as extra-small-bowel T-cell lymphomas; such as subcutaneous panniculitis-like lymphoma, hepatosplenic gamma/delta lymphoma, nodal as well as sinus, gastric or colon disease and extraintestinal T-cell lymphomas.
  • The majority of EATLs present as large cell lymphoma CD3+, CD8-, CD30+; however, they also present as small cell lymphoma CD3+, CD8+, CD30-.
  • Sometimes gamma/delta lymphomas in CD are recognized.
  • Work-up of EATL must include immunohistology, T-cell flow cytometry, T-cell rearrangement and adequate imaging with CT and PET scanning.
  • [MeSH-major] Celiac Disease / complications. Intestinal Neoplasms / diagnosis. Intestinal Neoplasms / therapy. Lymphoma, T-Cell / diagnosis. Lymphoma, T-Cell / therapy

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  • (PMID = 15696854.001).
  • [ISSN] 0085-5928
  • [Journal-full-title] Scandinavian journal of gastroenterology. Supplement
  • [ISO-abbreviation] Scand. J. Gastroenterol. Suppl.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Norway
  • [Number-of-references] 45
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27. Iannitto E, Barbera V, Quintini G, Cirrincione S, Leone M: Hepatosplenic gammadelta T-cell lymphoma: complete response induced by treatment with pentostatin. Br J Haematol; 2002 Jun;117(4):995-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hepatosplenic gammadelta T-cell lymphoma: complete response induced by treatment with pentostatin.
  • [MeSH-major] Immunosuppressive Agents / therapeutic use. Liver Neoplasms / drug therapy. Lymphoma, T-Cell / drug therapy. Pentostatin / therapeutic use. Receptors, Antigen, T-Cell, gamma-delta. Splenic Neoplasms / drug therapy
  • [MeSH-minor] Adult. Combined Modality Therapy. Hematopoietic Stem Cell Transplantation. Humans. Male

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  • (PMID = 12060145.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunosuppressive Agents; 0 / Receptors, Antigen, T-Cell, gamma-delta; 395575MZO7 / Pentostatin
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28. Jaeger G, Bauer F, Brezinschek R, Beham-Schmid C, Mannhalter C, Neumeister P: Hepatosplenic gammadelta T-cell lymphoma successfully treated with a combination of alemtuzumab and cladribine. Ann Oncol; 2008 May;19(5):1025-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hepatosplenic gammadelta T-cell lymphoma successfully treated with a combination of alemtuzumab and cladribine.

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  • (PMID = 18375525.001).
  • [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] Case Reports; Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antibodies, Neoplasm; 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / Receptors, Antigen, T-Cell, gamma-delta; 3A189DH42V / alemtuzumab; 47M74X9YT5 / Cladribine; 4F4X42SYQ6 / Rituximab; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; 9PHQ9Y1OLM / Prednisolone
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