T-cell large granular lymphocytic (T-LGL) leukemia: a single institution experience |
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Authors: | Aleksandra Sretenovic Darko Antic Snezana Jankovic Mirjana Gotic Maja Perunicic-Jovanovic Ljubomir Jakovic Biljana Mihaljevic |
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Institution: | (1) Institute of Hematology, Clinical Center Serbia, Koste Todorovica 2 Street, 11 000 Belgrade, Serbia; |
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Abstract: | 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. |
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