Aggressive T-cell large granular lymphocyte leukemia: a case report and review of the literature.

The majority of patients with T-cell large granular lymphocyte (LGL) leukemia will have an indolent clinical course. Herein, we report a case of an aggressive T-cell LGL leukemia in a previously healthy 42-year-old Caucasian male who presented with acute onset of B-symptoms, hepatosplenomegaly, lymphocytosis, moderate anemia, and thrombocytopenia. Immunophenotypically, the malignant cells co-expressed CD3(+)CD8(+)CD56(+) markers and the T-cell receptor beta (TCR beta) gene demonstrated clonal rearrangement. The patient was treated with an intensive chemotherapeutic regimen (hyper-CVAD) and he achieved a complete remission. A systematic review of all available English literature revealed 12 well-described cases of aggressive T-cell LGL leukemia suggesting that this variant is a new and distinct entity in the spectrum of LGL disorders. (c) 2007 Wiley-Liss, Inc.