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1. Pawarode A, Wallace PK, Ford LA, Barcos M, Baer MR: Long-term safety and efficacy of cyclosporin A therapy for T-cell large granular lymphocyte leukemia. Leuk Lymphoma; 2010 Feb;51(2):338-41
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  • [Title] Long-term safety and efficacy of cyclosporin A therapy for T-cell large granular lymphocyte leukemia.
  • [MeSH-major] Cyclosporine / therapeutic use. Leukemia, Large Granular Lymphocytic / drug therapy
  • [MeSH-minor] Aged. Aged, 80 and over. Humans. Immunosuppressive Agents / therapeutic use. Middle Aged. Neutropenia / complications. Neutropenia / drug therapy. Retrospective Studies. Time Factors. Treatment Outcome

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  • (PMID = 20038217.001).
  • [ISSN] 1029-2403
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunosuppressive Agents; 83HN0GTJ6D / Cyclosporine
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2. Dearden CE, Matutes E, Catovsky D: Alemtuzumab in T-cell malignancies. Med Oncol; 2002;19 Suppl:S27-32
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  • [Title] Alemtuzumab in T-cell malignancies.
  • Alemtuzumab is a humanized monoclonal antibody directed against the CD52 antigen, which is abundantly expressed on all normal and most malignant T-lymphocytes.
  • We summarize the results of our experience using alemtuzumab to treat a range of clinically aggressive, mature, post-thymic, T-cell malignancies, including T-cell prolymphocytic leukemia (T-PLL), cutaneous T-cell lymphoma (CTCL), T-cell large granular lymphocyte (T-LGL) leukemia, and human T-cell lymphotropic virus I (HTLV-I) associated adult T-cell leukemia-lymphoma (ATLL).
  • Alemtuzumab was administered at a dose of 30 mg, three times a week until maximum response.
  • Apart from first-dose reactions, which were common, treatment was well tolerated, the main complication being infection and viral reactivation associated with the prolonged lymphopenia.
  • Experience in T-LGL and ATLL is limited to single cases only and further studies are required to better define the role of alemtuzumab in these subgroups.
  • Our results indicate that alemtuzumab has activity in T-cell malignancies, particularly in T-PLL and in patients with predominantly blood and bone marrow disease.
  • It may be possible to prolong response duration by the use of high-dose therapy and stem cell transplantation.
  • Alemtuzumab may also have a role in purging minimal residual disease following other chemotherapy and prior to transplantation.
  • We conclude that treatment with alemtuzumab may offer new hope to patients who otherwise have a bleak prognosis.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antibodies, Neoplasm / therapeutic use. Antigens, CD / drug effects. Antigens, Neoplasm / drug effects. Antineoplastic Agents / therapeutic use. Glycoproteins / drug effects. Lymphoma, T-Cell / drug therapy

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  • (PMID = 12180489.001).
  • [ISSN] 1357-0560
  • [Journal-full-title] Medical oncology (Northwood, London, England)
  • [ISO-abbreviation] Med. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [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
  • [Number-of-references] 18
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3. Olteanu H, Harrington AM, Ramirez S, Kroft SH, Hari P: Efficacy and safety of long-term (>7 year) alemtuzumab therapy for refractory T-cell large granular lymphocytic leukaemia. Br J Haematol; 2010 Aug;150(4):480-1
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  • [Title] Efficacy and safety of long-term (>7 year) alemtuzumab therapy for refractory T-cell large granular lymphocytic leukaemia.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antibodies, Neoplasm / therapeutic use. Antineoplastic Agents / therapeutic use. Leukemia, Large Granular Lymphocytic / drug therapy
  • [MeSH-minor] Antibodies, Monoclonal, Humanized. Drug Administration Schedule. Female. Humans. Middle Aged. Purpura, Thrombocytopenic, Idiopathic / chemically induced

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  • (PMID = 20456357.001).
  • [ISSN] 1365-2141
  • [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 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antineoplastic Agents; 3A189DH42V / alemtuzumab
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4. Manucha V, Zhao F, Rodgers W: Atypical lymphoid cells in cerebrospinal fluid in acute Epstein Barr virus infection: a case report demonstrating a pitfall in cerebrospinal fluid cytology. Acta Cytol; 2008 May-Jun;52(3):334-6
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  • [Title] Atypical lymphoid cells in cerebrospinal fluid in acute Epstein Barr virus infection: a case report demonstrating a pitfall in cerebrospinal fluid cytology.
  • A well-recognized pitfall is overinterpretation of the presence of atypical lymphocytes that resemble malignant lymphoid cells in the CSF.
  • CASE: A 25-year-old patient with a past history of treated large granular lymphocytic leukemia and presence of a predominant population of large, atypical lymphoid cells in the CSF, giving us the impression of involvement with large cell lymphoma.
  • CONCLUSION: The presence of atypical cells in the CSF certainly warrants a detailed look at the patient's laboratory investigations and communication with the hematologist, because it may be the only specimen available for diagnosis on which therapy and prognosis is based.
  • [MeSH-major] Cerebrospinal Fluid / cytology. Cytological Techniques / methods. Epstein-Barr Virus Infections / cerebrospinal fluid. Epstein-Barr Virus Infections / pathology. Lymphocytes / pathology
  • [MeSH-minor] Adult. DNA, Viral / analysis. DNA, Viral / blood. Hepatomegaly / ultrasonography. Humans. Leukemia, Large Granular Lymphocytic / drug therapy. Lymph Nodes / pathology. Lymph Nodes / radiography. Lymphatic Diseases / pathology. Lymphatic Diseases / radiography. Male. Pleural Effusion / radiography. Splenectomy

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  • (PMID = 18540300.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Viral
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5. Sretenovic A, Antic D, Jankovic S, Gotic M, Perunicic-Jovanovic M, Jakovic L, Mihaljevic B: T-cell large granular lymphocytic (T-LGL) leukemia: a single institution experience. Med Oncol; 2010 Jun;27(2):286-90
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  • [Title] T-cell large granular lymphocytic (T-LGL) leukemia: a single institution experience.
  • BACKGROUND: T-cell large granular lymphocytic (T-LGL) leukemia is a rare lymphoproliferative disease which usually affects elderly people.
  • The clinical course of T-LGL leukemia is generally indolent, with lymphocytosis and splenomegaly in 20-50% patients, hepatomegaly in 5-20% of patients, and less commonly, lymphadenopathy.
  • T-LGL leukemia is associated with immunological abnormalities: rheumatoid factor with or without rheumatoid arthritis (RA), Coombs positive hemolytic anemia, idiopathic thrombocytopenic purpura (ITP), pure red cell aplasia (PRCA), positive anti-nuclear antibodies (ANA), anti-neutrophil cytoplasmic antibodies (ANCA), hypogammaglobulinemia, and polyclonal hypergammaglobulinemia.
  • Aim To compare clinical and laboratory features of T-LGL leukemia patients and their responses to different chemotherapy regimens.
  • METHODS: Six patients (3 males and 3 females) with T-LGL leukemia were analyzed.
  • The diagnosis was based on accepted morphologic criteria, immunophenotype, and polymerase chain reaction (PCR) detection of T-cell receptor (TCR) gene rearrangements.
  • RESULTS: All patients exhibited lymphocytosis, mainly with unusual morphologies, splenomegaly, and elevated serum lactate dehydrogenase (LDH).
  • Three patients were treated with a Fludarabine-Cyclophosphamide (FC) combination as initial therapy while three patients received CHOP.
  • Two patients received more than one treatment regimen.
  • One patient died due to T-LGL leukemia in first year after diagnosis, one patient died 4 years after diagnosis, two patients interrupted their treatment, and two patients are still alive.
  • CONCLUSIONS: Further prospective studies are needed for establishing a gold standard therapy for T-LGL leukemia.
  • [MeSH-major] Leukemia, Large Granular Lymphocytic / blood. Leukemia, Large Granular Lymphocytic / drug therapy
  • [MeSH-minor] Adult. Aged. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cyclophosphamide / therapeutic use. Doxorubicin / therapeutic use. Female. Follow-Up Studies. Humans. Male. Middle Aged. Prednisone / therapeutic use. Vidarabine / analogs & derivatives. Vidarabine / therapeutic use. Vincristine / therapeutic use

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  • (PMID = 19306076.001).
  • [ISSN] 1559-131X
  • [Journal-full-title] Medical oncology (Northwood, London, England)
  • [ISO-abbreviation] Med. Oncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; FA2DM6879K / Vidarabine; P2K93U8740 / fludarabine; VB0R961HZT / Prednisone; CHOP protocol
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6. Haran MZ, Basous L, Berrebi A: Multiple myeloma associated with CD4+ large granular lymphocytic leukemia: a possible causal relationship. Hematol J; 2004;5(5):458-60
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  • [Title] Multiple myeloma associated with CD4+ large granular lymphocytic leukemia: a possible causal relationship.


7. Osuji N, Matutes E, Tjonnfjord G, Grech H, Del Giudice I, Wotherspoon A, Swansbury JG, Catovsky D: T-cell large granular lymphocyte leukemia: A report on the treatment of 29 patients and a review of the literature. Cancer; 2006 Aug 1;107(3):570-8
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  • [Title] T-cell large granular lymphocyte leukemia: A report on the treatment of 29 patients and a review of the literature.
  • BACKGROUND: To the authors' knowledge, there is no standard treatment for patients with T-cell large granular lymphocyte (LGL) leukemia.
  • METHODS: The authors report on the use of immunosuppressants (cyclosporin A [CSA] and low-dose oral methotrexate [MTX] given continuously) and cytotoxic agents in the treatment of 29 patients with T-cell LGL leukemia age over the past 20 years.
  • The median time to response for both agents was 1 month.
  • An ORR of 67% (all CHR) was attained with pentostatin (n = 4 patients); recurrences developed after a median of 4.6 years.
  • Successful retreatment with pentostatin was possible but with increasing drug resistance.
  • Alemtuzumab induced a PR in 1 patient who had refractory disease.
  • CONCLUSIONS: Both MTX and CSA were efficacious in the treatment of T-cell LGL leukemia but generally required long-term maintenance therapy.
  • The authors highlight the risks of second malignancies and persistence of bone marrow disease.
  • Although MTX and CSA were effective as first-line therapy, alemtuzumab and pentostatin merit further investigation, particularly for refractory disease.
  • [MeSH-major] Immunosuppressive Agents / therapeutic use. Leukemia, Lymphoid / drug therapy. Leukemia, T-Cell / drug therapy
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Cyclophosphamide / therapeutic use. Cyclosporine / therapeutic use. Databases as Topic. Female. Humans. Male. Methotrexate / therapeutic use. Middle Aged. Pentostatin / therapeutic use. Retrospective Studies


8. Takahashi T, Otani I, Okuda M, Inoue M, Ito K, Sakai M, Koie H, Yamaya Y, Watari T, Sato T, Kanayama K, Tokuriki M: Malignant transformation of T-cell large granular lymphocyte leukemia in a dog. J Vet Med Sci; 2007 Jun;69(6):677-81
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  • [Title] Malignant transformation of T-cell large granular lymphocyte leukemia in a dog.
  • An 8-year-old spayed female Golden Retriever was referred to us for evaluation of mild lymphocytosis.
  • The peripheral lymphocytes were comprised of mostly large granular lymphocytes (LGLs), and flow cytometry showed that they were mostly CD3+8+ T lymphocytes.
  • Clonal rearrangement of the T-cell receptor gene was identified in the peripheral blood, and the dog was therefore diagnosed with LGL chronic leukemia.
  • The dog was subclinical without treatment until hospitalization on day 154, at which point the lymphocytes looked like lymphoblasts and the surface markers changed to CD3-8-.
  • This was regarded as malignant transformation from LGL chronic leukemia to the acute type.
  • Sequential chemotherapy was started, but the dog died on day 190.
  • Necropsy revealed tumor cell infiltration into the heart, skin, and brain.

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  • (PMID = 17611371.001).
  • [ISSN] 0916-7250
  • [Journal-full-title] The Journal of veterinary medical science
  • [ISO-abbreviation] J. Vet. Med. Sci.
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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9. Christopoulos PD, Katsoudas S, Androulaki A, Nakopoulou L, Economopoulos T, Vlahakos DV: T-cell large granular lymphocyte leukemia presenting as end-stage renal disease: the diagnostic role of flow-cytometric and clonality analysis of the urine sediment. Clin Nephrol; 2009 Feb;71(2):198-202
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  • [Title] T-cell large granular lymphocyte leukemia presenting as end-stage renal disease: the diagnostic role of flow-cytometric and clonality analysis of the urine sediment.
  • Several liver and bone marrow biopsies during that period had shown a nonspecific polyclonal T-cell infiltration, and she was administered low-dose steroids for symptomatic relief.
  • Physical examination, laboratory workup and imaging studies at presentation showed pancytopenia, hepatosplenomegaly, large symmetric kidneys with normal cortices and no evidence of obstructive uropathy, aseptic pyuria with neutrophils and lymphocytes and mild proteinuria.
  • On biopsy the renal interstitium was infiltrated by large, granular CD3+CD8+CD56-CD57+ lymphocytes, clonal by molecular analysis, which established the diagnosis of T-cell large granular lymphocyte leukemia.
  • Most urinary and peripheral blood lymphocytes bore the same T-LGL surface markers and were also clonal, as shown by flow-cytometry and PCR amplification of the T-cell receptor g-chain genes.
  • After exclusion of an underlying EBV, CMV, HBV, HCV or HIV infection with negative serology and blood PCR the patient received one cycle of chemotherapy with cyclophosphamide, vincristine and prednisone.
  • No improvement of renal function was achieved, while complication with a prolonged pulmonary infection and severe sepsis precluded further treatment.
  • Our report indicates that the T-LGL leukemia should be considered in the differential diagnosis of renal failure with large-sized kidneys, especially when hepatosplenomegaly, pancytopenia and aseptic pyuria are also present.
  • Since renal function may deteriorate rapidly, chemotherapy should not be delayed.
  • [MeSH-major] Flow Cytometry. Kidney Failure, Chronic / diagnosis. Leukemia, Large Granular Lymphocytic / diagnosis
  • [MeSH-minor] Aged. Diagnosis, Differential. Female. Humans. Tomography, X-Ray Computed


10. Go RS, Tefferi A, Li CY, Lust JA, Phyliky RL: Lymphoproliferative disease of granular T lymphocytes presenting as aplastic anemia. Blood; 2000 Nov 15;96(10):3644-6
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  • [Title] Lymphoproliferative disease of granular T lymphocytes presenting as aplastic anemia.
  • Lymphoproliferative disease of granular T lymphocyte (T-LDGL), also known as T-cell large granular lymphocyte leukemia, is a clonal disorder of cytotoxic T lymphocytes that is clinically manifested as chronic neutropenia and anemia.
  • Morphologic evidence of increased granular lymphocytes in the peripheral blood and an excess of CD3(+)/CD8(+)/CD57(+) cells in the bone marrow were found in most cases.
  • After a median follow-up of 49 months, 5 patients had died from the disease or related complications.
  • [MeSH-major] Anemia, Aplastic / etiology. Leukemia, T-Cell / diagnosis
  • [MeSH-minor] Adult. Aged. Bone Marrow Cells / immunology. Cyclophosphamide / administration & dosage. Cyclophosphamide / standards. Cytogenetic Analysis. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Immunophenotyping. Immunosuppressive Agents / administration & dosage. Immunosuppressive Agents / standards. Leukemia, Lymphoid / complications. Leukemia, Lymphoid / diagnosis. Leukemia, Lymphoid / drug therapy. Lymphoproliferative Disorders / complications. Lymphoproliferative Disorders / diagnosis. Lymphoproliferative Disorders / drug therapy. Male. Middle Aged. Prednisone / administration & dosage. Prednisone / standards. Survival Rate. Treatment Outcome

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  • (PMID = 11071666.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Immunosuppressive Agents; 8N3DW7272P / Cyclophosphamide; VB0R961HZT / Prednisone
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11. Malani AK, Gupta C, Rangineni R, Singh J, Ammar H: Concomitant presentation of acute myeloid leukemia with T-cell large granular lymphocytic leukemia. Acta Oncol; 2007;46(2):247-9
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  • [Title] Concomitant presentation of acute myeloid leukemia with T-cell large granular lymphocytic leukemia.
  • T-cell large granular lymphocyte leukemia (T-LGL) also known as T-cell chronic lymphocytic leukemia is rare and comprises a small minority of all small lymphocytic leukemias.
  • The concomitant presentation of T-LGL with acute myeloid leukemia (AML) has not been previously reported.
  • We present an elderly gentleman with concomitant T-LGL and AML (non-M3) diagnosed by a combination of morphologic evaluation, immunophenotyping by flow cytometry, and T-cell gene rearrangement studies.
  • The patient was managed with combination AML chemotherapy.
  • [MeSH-major] Leukemia, Myeloid / diagnosis. Leukemia, Prolymphocytic, T-Cell / diagnosis
  • [MeSH-minor] Acute Disease. Aged, 80 and over. Antigens, CD / analysis. Flow Cytometry. Humans. Male

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  • (PMID = 17453377.001).
  • [ISSN] 0284-186X
  • [Journal-full-title] Acta oncologica (Stockholm, Sweden)
  • [ISO-abbreviation] Acta Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Norway
  • [Chemical-registry-number] 0 / Antigens, CD
  • [Number-of-references] 17
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12. Mohan SR, Clemente MJ, Afable M, Cazzolli HN, Bejanyan N, Wlodarski MW, Lichtin AE, Maciejewski JP: Therapeutic implications of variable expression of CD52 on clonal cytotoxic T cells in CD8+ large granular lymphocyte leukemia. Haematologica; 2009 Oct;94(10):1407-14
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  • [Title] Therapeutic implications of variable expression of CD52 on clonal cytotoxic T cells in CD8+ large granular lymphocyte leukemia.
  • BACKGROUND: T-cell large granular lymphocytic leukemia is a clonal proliferation of cytotoxic T-lymphocytes which often results in severe cytopenia.
  • Current treatment options favor chronic immunosuppression.
  • Alemtuzumab, a humanized monoclonal antibody against glycophosphatidylinositol-anchored CD52, is approved for patients refractory to therapy in other lymphoid malignancies.
  • DESIGN AND METHODS: We retrospectively examined treatment outcomes in 59 patients with CD8+ T-cell large granular lymphocytic leukemia, 41 of whom required therapy.
  • Eight patients with severe refractory cytopenia despite multiple treatment regimens had been treated with subcutaneous alemtuzumab as salvage therapy.
  • Flow cytometry was used to monitor expression of glycophosphatidylinositol-anchored CD52, CD55, and CD59 as well as to characterize T-cell clonal expansions by T-cell receptor variable beta-chain (Vbeta) repertoire.
  • RESULTS: Analysis of the effects of alemtuzumab revealed remissions with restoration of platelets in one of one patient, red blood cell transfusion independence in three of five patients and improvement of neutropenia in one of three, resulting in an overall response rate of 50% (4/8 patients).
  • Clonal large granular lymphocytes exhibited decreased CD52 expression post-therapy in patients refractory to treatment.
  • Samples of large granular lymphocytes collected prior to therapy also unexpectedly had a significant proportion of CD52-negative cells while a healthy control population had no such CD52 deficiency (p=0.026).
  • CONCLUSIONS: While alemtuzumab may be highly effective in large granular lymphocytic leukemia, prospective serial monitoring for the presence of CD52-deficient clonal cytotoxic T-lymphocytes should be a component of clinical trials investigating the efficacy of this drug.
  • CD52 deficiency may explain lack of response to alemtuzumab, and such therapy may confer a survival advantage to glycophosphatidylinositol-negative clonal cytotoxic T-lymphocytes.

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  • (PMID = 19794084.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA113972; United States / NCRR NIH HHS / RR / U54 RR019397; United States / NCRR NIH HHS / RR / U54 RR 019397
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antigens, CD; 0 / Antigens, Neoplasm; 0 / CD52 antigen; 0 / Glycoproteins; 3A189DH42V / alemtuzumab
  • [Other-IDs] NLM/ PMC2754957
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13. Fortune AF, Kelly K, Sargent J, O'Brien D, Quinn F, Chadwick N, Flynn C, Conneally E, Browne P, Crotty GM, Thornton P, Vandenberghe E: Large granular lymphocyte leukemia: natural history and response to treatment. Leuk Lymphoma; 2010 May;51(5):839-45
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  • [Title] Large granular lymphocyte leukemia: natural history and response to treatment.
  • Large granular lymphocyte leukemia (T-LGL) is an indolent T lymphoproliferative disorder that was difficult to diagnose with certainty until clonality testing of the T cell receptor gene became routinely available.
  • We studied the natural history and response to treatment in 25 consecutive patients with T-LGL diagnosed between 2004 and 2008 in which the diagnosis was confirmed by molecular analysis, to define an effective treatment algorithm.
  • The median age at diagnosis was 61 years (range 27-78), with a male to female ratio of 1:1.8 and presenting features of fatigue (n = 13), recurrent infections (n = 9), and/or abnormal blood counts (n = 5).
  • Thirteen patients with symptomatic disease were treated as follows: pentostatin (nine patients), cyclosporine (six patients), methotrexate (three patients), and alemtuzumab in two patients in whom pentostatin was ineffective.
  • Pentostatin was the single most effective therapy, with a response rate of 75% and minimal toxicity.
  • Treatment of T-LGL should be reserved for patients with symptomatic disease, but in this series, pentostatin treatment was less toxic and more effective than cyclosporine or methotrexate.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cyclosporine / therapeutic use. Immunosuppressive Agents / therapeutic use. Leukemia, Large Granular Lymphocytic / drug therapy
  • [MeSH-minor] Adult. Aged. Antibodies, Monoclonal / administration & dosage. Antibodies, Monoclonal, Humanized. Antibodies, Neoplasm / administration & dosage. Drug Therapy, Combination. Female. Humans. Male. Methotrexate / administration & dosage. Middle Aged. Pentostatin / administration & dosage. Survival Rate. Treatment Outcome

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  • (PMID = 20367569.001).
  • [ISSN] 1029-2403
  • [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 / Immunosuppressive Agents; 395575MZO7 / Pentostatin; 3A189DH42V / alemtuzumab; 83HN0GTJ6D / Cyclosporine; YL5FZ2Y5U1 / Methotrexate
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14. Moosig F, Schoch R, Kneba M: [T-large granular lymphocyte leukaemia. An important differential diagnosis to Felty's syndrome]. Z Rheumatol; 2006 Sep;65(5):447-51
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  • [Title] [T-large granular lymphocyte leukaemia. An important differential diagnosis to Felty's syndrome].
  • [Transliterated title] Die T-large Granular Lymphocyte Leukämie (T-LGL-Leukämie). Eine wichtige Differenzialdiagnose zum Felty-syndrom.
  • T-Large Granular Lymphocyte (T-LGL) leukaemia is a rare clonal disease characterized by neutropenia and/or anaemia.
  • Because of its strong association with rheumatoid arthritis (RA), T-LGL leukaemia is an important differential diagnosis to Felty's syndrome.
  • This differentiation might be especially difficult since, in severe RA with extraarticular manifestations, there is often an expanded memory effector T-cell population which can hardly be separated from T-LGL leukaemia cells by means of immunophenotyping.
  • The main criterion for T-LGL leukaemia is the detection of a clonal T-cell-receptor rearrangement by PCR.
  • First-line therapy consists of weekly low-dose methotrexate.
  • There are very limited data from therapy studies.
  • The German CLL study group has initiated a protocol using parenteral low-dose methotrexate as first-line therapy and fludarabine as second-line medication.
  • [MeSH-major] Leukemia, Lymphoid / diagnosis. Leukemia, T-Cell / diagnosis
  • [MeSH-minor] Antirheumatic Agents / therapeutic use. Antiviral Agents / therapeutic use. Arthritis, Rheumatoid / diagnosis. Arthritis, Rheumatoid / drug therapy. Diagnosis, Differential. Felty Syndrome / diagnosis. Humans. Immunosuppressive Agents / therapeutic use. Methotrexate / therapeutic use. Vidarabine / analogs & derivatives. Vidarabine / therapeutic use

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  • (PMID = 16450150.001).
  • [ISSN] 0340-1855
  • [Journal-full-title] Zeitschrift fur Rheumatologie
  • [ISO-abbreviation] Z Rheumatol
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antirheumatic Agents; 0 / Antiviral Agents; 0 / Immunosuppressive Agents; FA2DM6879K / Vidarabine; P2K93U8740 / fludarabine; YL5FZ2Y5U1 / Methotrexate
  • [Number-of-references] 39
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15. Cheung MM, Chan JK, Wong KF: Natural killer cell neoplasms: a distinctive group of highly aggressive lymphomas/leukemias. Semin Hematol; 2003 Jul;40(3):221-32
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  • [Title] Natural killer cell neoplasms: a distinctive group of highly aggressive lymphomas/leukemias.
  • Natural killer (NK) cell neoplasms, which include extranodal NK/T-cell lymphoma (nasal and extranasal) and aggressive NK cell leukemia, are generally rare, but they are more common in people of Oriental, Mexican and South American descent.
  • Extranodal NK/T-cell lymphoma most commonly affects the nasal cavity and other mucosal sites of the upper aerodigestive tract.
  • Despite the early stage of disease at presentation, overall survival is poor.
  • Patients with the extranasal form of the lymphoma often present with high-stage disease, commonly involving the skin, gastrointestinal tract, testis, and soft tissue, and the prognosis is even worse.
  • Based on currently available data, treatment of nasal NK/T-cell lymphoma should consist of radiotherapy, with or without multiagent chemotherapy.
  • More research is required to ascertain the role of high-dose chemotherapy with stem cell rescue and that of non-multidrug resistance-related chemotherapeutic agents.
  • Aggressive NK cell leukemia 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.
  • The peripheral blood and bone marrow show atypical large granular lymphocytes, which exhibit an immunophenotype similar to that of extranodal NK/T-cell lymphoma.
  • Aggressive NK cell leukemia must be distinguished from T-cell large granular lymphocyte leukemia and indolent NK cell lymphoproliferative disorder, both of which are indolent.
  • [MeSH-major] Killer Cells, Natural / pathology. Leukemia / pathology. Lymphoma / pathology
  • [MeSH-minor] Diagnosis, Differential. Humans. Lymphoma, T-Cell / diagnosis. Lymphoma, T-Cell / pathology. Lymphoma, T-Cell / therapy. Treatment Outcome

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  • (PMID = 12876671.001).
  • [ISSN] 0037-1963
  • [Journal-full-title] Seminars in hematology
  • [ISO-abbreviation] Semin. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 99
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16. Sokol L, Loughran TP Jr: Large granular lymphocyte leukemia and natural killer cell leukemia/lymphomas. Curr Treat Options Oncol; 2003 Aug;4(4):289-96
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  • [Title] Large granular lymphocyte leukemia and natural killer cell leukemia/lymphomas.
  • Natural killer (NK) cell leukemia and lymphoma represent rare conditions with heterogeneity of biologic behavior, prognosis, and responsiveness to therapy.
  • The initial diagnosis of NK-cell malignancies can be difficult because of the lack of immunophenotypic clonality markers, morphologic heterogeneity, and a poor correlation between cytomorphology and prognosis.
  • Therapeutic recommendations for NK-cell malignancies are derived from retrospective studies or case reports.
  • Immature NK-cell malignancies often have aggressive behavior with poor prognosis, despite administration of acute myeloid leukemia or acute lymphocytic leukemia induction chemotherapy.
  • The use of high-dose chemotherapy with stem cell rescue resulted in a prolonged survival in a small series of patients.
  • NK-cell malignancies originating from cells with mature phenotypes form a spectrum of diseases with distinct prognosis.
  • Patients with aggressive NK-cell leukemia invariably die within several months.
  • Nasal and nasal-like NK/T-cell lymphomas with limited stage disease often respond to radiation therapy alone or combination with chemotherapy and radiation therapy, with 5-year disease-free survival rates ranging from 30% to 75%.
  • Patients with T-cell large granular lymphocyte leukemia or chronic NK-cell lymphoproliferative disease of granular lymphocytes can have an indolent clinical course with long survival without therapy.
  • However, approximately 66% of patients with T-cell large granular lymphocyte leukemia require low-dose chemotherapy with methotrexate or cyclophosphamide or immunosuppressive therapy with glucocorticosteroids or cyclosporine A for symptomatic cytopenias during the course of their disease.
  • [MeSH-major] Killer Cells, Natural. Leukemia / therapy. Lymphoma / therapy. Precursor Cell Lymphoblastic Leukemia-Lymphoma / therapy


17. Lamy T, Loughran TP Jr: Clinical features of large granular lymphocyte leukemia. Semin Hematol; 2003 Jul;40(3):185-95
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  • [Title] Clinical features of large granular lymphocyte leukemia.
  • The spectrum of large granular lymphocyte (LGL) proliferations consists of four distinct entities: reactive/transient LGL expansion, chronic LGL lymphocytosis, classical indolent LGL leukemia, and aggressive LGL leukemia.
  • LGL leukemias are classified as lymphoid malignancies.
  • They are divided into CD3(+)/T-cell LGL (85% of cases) and CD3(-)/natural killer (NK) cell LGL leukemia (15% of cases).
  • Identification of LGL expansion has been improved using T-cell receptor (TCR)beta/gamma polymerase chain reaction (PCR) analysis and a combination of Vbeta and killer cell immunoglobulin-like receptor (KIR)-specific monoclonal antibodies.
  • LGL leukemias are characterized by a clonal LGL infiltration of the bone marrow, spleen, and liver.
  • T-LGL leukemias affect the elderly and display a relatively indolent behavior.
  • Approximately 60% to 70% of patients are symptomatic: recurrent infections secondary to chronic neutropenia, anemia, and autoimmune disease such as rheumatoid arthritis are the main clinical manifestations.
  • Conversely, NK LGL leukemias behave aggressively, and most patients do not respond to chemotherapy.
  • [MeSH-major] Leukemia, Lymphoid / pathology
  • [MeSH-minor] Autoimmune Diseases. Clone Cells / immunology. Clone Cells / pathology. Hematologic Diseases. Humans. Killer Cells, Natural / immunology. Killer Cells, Natural / pathology. T-Lymphocytes / immunology. T-Lymphocytes / pathology


18. 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.
  • 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.
  • Novel targeted therapies are currently being tested in clinical studies.
  • [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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19. Dearden C: The role of alemtuzumab in the management of T-cell malignancies. Semin Oncol; 2006 Apr;33(2 Suppl 5):S44-52
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The role of alemtuzumab in the management of T-cell malignancies.
  • T-cell malignancies are rare, making up 10% to 15% of all lymphoid neoplasms in adults.
  • They include many different types of disorders such as T-cell prolymphocytic leukemia, T-cell large granular lymphocytic leukemia, adult T-cell leukemia/lymphoma, cutaneous T-cell lymphoma, and peripheral T-cell lymphoma, which are themselves divided into multiple subcategories.
  • Most T-cell malignancies arise as a result of chromosomal abnormalities, including T-cell receptor rearrangement anomalies.
  • Viral infections are implicated in the development of adult T-cell leukemia/lymphoma and some cases of peripheral T-cell lymphoma have been linked to Epstein-Barr virus or human immunodeficiency virus infection.
  • With the possible exception of T-cell large granular lymphocytic leukemia, which often has an indolent course, T-cell malignancies have not responded well to conventional chemotherapeutic treatment.
  • The introduction of monoclonal antibodies for the treatment of cancer has changed the outlook for patients with T-cell malignancies.
  • Recent studies with single-agent alemtuzumab, an anti-CD52 monoclonal antibody, have shown improved response rates and survival in patients with T-cell prolymphocytic leukemia and cutaneous T-cell lymphoma.
  • Preliminary data also suggest that alemtuzumab may have activity in patients with heavily pretreated peripheral T-cell lymphoma who are refractory to conventional chemotherapy.
  • Preclinical studies with mice bearing human adult T-cell leukemia/lymphoma cells suggest that alemtuzumab may have a potential therapeutic role in this setting.
  • Treatment of T-cell hematologic malignancies with alemtuzumab appears promising.
  • Earlier treatment and combination with chemotherapeutic agents may improve treatment outcome for patients with these malignancies and allow for consolidation with stem cell transplant strategies in selected patients.
  • [MeSH-major] Antibodies, Monoclonal / therapeutic use. Antibodies, Neoplasm / therapeutic use. Antineoplastic Agents / therapeutic use. Leukemia, T-Cell / drug therapy. Lymphoma, T-Cell / drug therapy
  • [MeSH-minor] Animals. Antibodies, Monoclonal, Humanized. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Humans. Neoadjuvant Therapy. Remission Induction. Stem Cell Transplantation. Survival Rate

  • MedlinePlus Health Information. consumer health - Cancer Chemotherapy.
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  • (PMID = 16720203.001).
  • [ISSN] 0093-7754
  • [Journal-full-title] Seminars in oncology
  • [ISO-abbreviation] Semin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antibodies, Neoplasm; 0 / Antineoplastic Agents; 3A189DH42V / alemtuzumab
  • [Number-of-references] 103
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