title: Aggressive T-cell large granular lymphocyte leukemia: a case report and review of the literature.
abstract: 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.
authors.affiliation: Division of Hematology and Oncology, H. Lee Moffitt Cancer Center and Research Institute, University of South Florida, Tampa, Florida, USA.
authors.forename: Todd J
authors.initials: TJ
authors.lastname: Alekshun
authors.forename: Jianguo
authors.initials: J
authors.lastname: Tao
authors.forename: Lubomir
authors.initials: L
authors.lastname: Sokol
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D018450:Disease Progression; D005500:Follow-Up Studies; D006801:Humans; D016130:Immunophenotyping; D007621:Karyotyping; D054066:Leukemia, Large Granular Lymphocytic / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D008297:Male; D012074:Remission Induction; D016896:Treatment Outcome