Large granular lymphocyte leukemia: natural history and response to treatment.

Hdl Handle:
http://hdl.handle.net/10147/207811
Title:
Large granular lymphocyte leukemia: natural history and response to treatment.
Authors:
Fortune, Anne F; Kelly, Kevin; Sargent, Jeremy; O'Brien, David; Quinn, Fiona; Chadwick, Nick; Flynn, Catherine; Conneally, Eibhlin; Browne, Paul; Crotty, Gerard M; Thornton, Patrick; Vandenberghe, Elisabeth
Affiliation:
National Adult Bone Marrow Transplant and Haematology Unit, St James Hospital,, Dublin 8, Ireland. afortune@stjames.ie
Citation:
Leuk Lymphoma. 2010 May;51(5):839-45.
Journal:
Leukemia & lymphoma
Issue Date:
1-Feb-2012
URI:
http://hdl.handle.net/10147/207811
DOI:
10.3109/10428191003706947
PubMed ID:
20367569
Abstract:
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. The overall survival (OS) and progression-free survival (PFS) 37 months from diagnosis were 80% and 52%, respectively. 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.
Language:
eng
MeSH:
Adult; Aged; Antibodies, Monoclonal/administration & dosage; Antibodies, Monoclonal, Humanized; Antibodies, Neoplasm/administration & dosage; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use; Cyclosporine/*therapeutic use; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents/*therapeutic use; Leukemia, Large Granular Lymphocytic/*drug therapy/pathology; Male; Methotrexate/administration & dosage; Middle Aged; Pentostatin/administration & dosage; Survival Rate; Treatment Outcome
ISSN:
1029-2403 (Electronic); 1026-8022 (Linking)

Full metadata record

DC FieldValue Language
dc.contributor.authorFortune, Anne Fen_GB
dc.contributor.authorKelly, Kevinen_GB
dc.contributor.authorSargent, Jeremyen_GB
dc.contributor.authorO'Brien, Daviden_GB
dc.contributor.authorQuinn, Fionaen_GB
dc.contributor.authorChadwick, Nicken_GB
dc.contributor.authorFlynn, Catherineen_GB
dc.contributor.authorConneally, Eibhlinen_GB
dc.contributor.authorBrowne, Paulen_GB
dc.contributor.authorCrotty, Gerard Men_GB
dc.contributor.authorThornton, Patricken_GB
dc.contributor.authorVandenberghe, Elisabethen_GB
dc.date.accessioned2012-02-01T10:45:23Z-
dc.date.available2012-02-01T10:45:23Z-
dc.date.issued2012-02-01T10:45:23Z-
dc.identifier.citationLeuk Lymphoma. 2010 May;51(5):839-45.en_GB
dc.identifier.issn1029-2403 (Electronic)en_GB
dc.identifier.issn1026-8022 (Linking)en_GB
dc.identifier.pmid20367569en_GB
dc.identifier.doi10.3109/10428191003706947en_GB
dc.identifier.urihttp://hdl.handle.net/10147/207811-
dc.description.abstractLarge 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. The overall survival (OS) and progression-free survival (PFS) 37 months from diagnosis were 80% and 52%, respectively. 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.en_GB
dc.language.isoengen_GB
dc.subject.meshAdulten_GB
dc.subject.meshAgeden_GB
dc.subject.meshAntibodies, Monoclonal/administration & dosageen_GB
dc.subject.meshAntibodies, Monoclonal, Humanizeden_GB
dc.subject.meshAntibodies, Neoplasm/administration & dosageen_GB
dc.subject.meshAntineoplastic Combined Chemotherapy Protocols/*therapeutic useen_GB
dc.subject.meshCyclosporine/*therapeutic useen_GB
dc.subject.meshDrug Therapy, Combinationen_GB
dc.subject.meshFemaleen_GB
dc.subject.meshHumansen_GB
dc.subject.meshImmunosuppressive Agents/*therapeutic useen_GB
dc.subject.meshLeukemia, Large Granular Lymphocytic/*drug therapy/pathologyen_GB
dc.subject.meshMaleen_GB
dc.subject.meshMethotrexate/administration & dosageen_GB
dc.subject.meshMiddle Ageden_GB
dc.subject.meshPentostatin/administration & dosageen_GB
dc.subject.meshSurvival Rateen_GB
dc.subject.meshTreatment Outcomeen_GB
dc.titleLarge granular lymphocyte leukemia: natural history and response to treatment.en_GB
dc.contributor.departmentNational Adult Bone Marrow Transplant and Haematology Unit, St James Hospital,, Dublin 8, Ireland. afortune@stjames.ieen_GB
dc.identifier.journalLeukemia & lymphomaen_GB
dc.description.provinceLeinster-

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