title: Large granular lymphocyte leukemia: natural history and response to treatment.
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.
authors.affiliation: National Adult Bone Marrow Transplant and Haematology Unit, St James Hospital, Dublin 8, Ireland. afortune@stjames.ie
authors.forename: Anne F
authors.initials: AF
authors.lastname: Fortune
authors.forename: Kevin
authors.initials: K
authors.lastname: Kelly
authors.forename: Jeremy
authors.initials: J
authors.lastname: Sargent
authors.forename: David
authors.initials: D
authors.lastname: O'Brien
authors.forename: Fiona
authors.initials: F
authors.lastname: Quinn
authors.forename: Nick
authors.initials: N
authors.lastname: Chadwick
authors.forename: Catherine
authors.initials: C
authors.lastname: Flynn
authors.forename: Eibhlin
authors.initials: E
authors.lastname: Conneally
authors.forename: Paul
authors.initials: P
authors.lastname: Browne
authors.forename: Gerard M
authors.initials: GM
authors.lastname: Crotty
authors.forename: Patrick
authors.initials: P
authors.lastname: Thornton
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Vandenberghe
mesh_terms: D000328:Adult; D000368:Aged; D000074323:Alemtuzumab; D000911:Antibodies, Monoclonal / Q000008:administration & dosage; D061067:Antibodies, Monoclonal, Humanized; D000912:Antibodies, Neoplasm / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016572:Cyclosporine / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D054066:Leukemia, Large Granular Lymphocytic / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D008875:Middle Aged; D015649:Pentostatin / Q000008:administration & dosage; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D000912:Antibodies, Neoplasm; D007166:Immunosuppressive Agents; D015649:Pentostatin; D000074323:Alemtuzumab; D016572:Cyclosporine; D008727:Methotrexate