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Aggressive T‐cell large granular lymphocyte leukemia: A case report and review of the literature
Author(s) -
Alekshun Todd J.,
Tao Jianguo,
Sokol Lubomir
Publication year - 2007
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.20853
Subject(s) - lymphocytosis , hepatosplenomegaly , leukemia , gene rearrangement , medicine , cd8 , immunology , t cell receptor , cd3 , t cell , pathology , biology , antigen , immune system , gene , biochemistry , disease
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.