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.
Sretenovic
Aleksandra
A
Institute of Hematology, Clinical Center Serbia, Koste Todorovica 2 Street, 11 000 Belgrade, Serbia.
Antic
Darko
D
Jankovic
Snezana
S
Gotic
Mirjana
M
Perunicic-Jovanovic
Maja
M
Jakovic
Ljubomir
L
Mihaljevic
Biljana
B
D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D054066:Leukemia, Large Granular Lymphocytic / Q000097:blood* / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D014740:Vidarabine / Q000031:analogs & derivatives / Q000627:therapeutic use; D014750:Vincristine / Q000627:therapeutic use
D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D014740:Vidarabine; C024352:fludarabine; D011241:Prednisone