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.
T-cell leukemia Large granular lymphocytic leukemia T-LGL leukemia Clinical data Chemotherapy
This is a preview of subscription content, log in to check access.
Chan WC, Catovsky D, Foucar K, Montserrat E. T-cell large granular lymphocyte leukaemia. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. Pathology and genetics of tumours of hepatopoietic and lymphoid tissues. IARC: Lyon; 2001. p. 197–8.Google Scholar
Dhodapkar M, Chin-Yang L, Lust J, Tefferi A, Phyliky RL. Clinical spectrum of clonal proliferations of T-large granular lymphocytes: a T-cell clonopathy of undetermined significance? Blood. 1994;84:1620–7.PubMedGoogle Scholar
Loughran TP Jr, Kidd PG, Starkebaum G. Treatment of large granular lymphocyte leukemia with oral low-dose methotrexate. Blood. 1994;84:2164–70.PubMedGoogle Scholar
Matrai Z, Lelkes G, Milosevits J, Paldine HP, Pecze K. T-cell large granular lymphocytic leukemia associated with pure red cell aplasia, successfully treated with cyclophosphamide. Orv Hetil. 1997;138:2075–80. (Hungarian).PubMedGoogle Scholar
Langford CA, Klippel JH, Balow JE, James SP, Sneller MC. Use of cytotoxic agents and cyclosporine in the treatment of autoimmune disease. Part 2. Inflammatory bowel disease and systemic vasculitis, and therapeutic toxicity. Ann Intern Med. 1998;129:49–58.PubMedGoogle Scholar