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 β) 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. Am. J. Hematol., 2007. © 2007 Wiley‐Liss, Inc.