title: T-cell large granular lymphocytic (T-LGL) leukemia: a single institution experience.
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.
authors.affiliation: Institute of Hematology, Clinical Center Serbia, Koste Todorovica 2 Street, 11 000 Belgrade, Serbia.
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Sretenovic
authors.forename: Darko
authors.initials: D
authors.lastname: Antic
authors.forename: Snezana
authors.initials: S
authors.lastname: Jankovic
authors.forename: Mirjana
authors.initials: M
authors.lastname: Gotic
authors.forename: Maja
authors.initials: M
authors.lastname: Perunicic-Jovanovic
authors.forename: Ljubomir
authors.initials: L
authors.lastname: Jakovic
authors.forename: Biljana
authors.initials: B
authors.lastname: Mihaljevic
mesh_terms: 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
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D014740:Vidarabine; C024352:fludarabine; D011241:Prednisone