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: Aleksandra Sretenovic, Darko Antic, Snezana Jankovic, Mirjana Gotic, Maja Perunicic-Jovanovic, Ljubomir Jakovic, Biljana Mihaljevic
• Journal: Medical oncology (Northwood, London, England) (Med Oncol) Vol. 27 Issue 2 Pg. 286-90 (Jun 2010) ISSN: 1559-131X [Electronic] United States
• PMID: 19306076 (Publication Type: Comparative Study, Journal Article)

Chemical References

• Vincristine
• Doxorubicin
• Cyclophosphamide
• Vidarabine
• fludarabine
• Prednisone

Topics

• Adult
• Aged
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Cyclophosphamide (therapeutic use)
• Doxorubicin (therapeutic use)
• Female
• Follow-Up Studies
• Humans
• Leukemia, Large Granular Lymphocytic (blood, diagnosis, drug therapy)
• Male
• Middle Aged
• Prednisone (therapeutic use)
• Vidarabine (analogs & derivatives, therapeutic use)
• Vincristine (therapeutic use)