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T-Cell Large Granular Lymphocyte Leukemia and Related Disorders

^a Yale University School of Medicine, The Comprehensive Cancer Center (IIID), VA Connecticut Healthcare System, West Haven, Connecticut, USA; ^b Section of Hematology, Yale University School of Medicine, New Haven, Connecticut, USA

Correspondence: Michal G. Rose, M.D., The Comprehensive Cancer Center (IIID), VA Connecticut Healthcare System, 950 Campbell Avenue, West Haven, Connecticut 06516, USA. Telephone: 203-937-3421; Fax: 203-937-3803; e-mail: Michal.Rose{at}med.va.gov, michalrose{at}sbcglobal.net

T-cell large granular lymphocyte (LGL) leukemia is a clonal proliferation of cytotoxic T cells, which causes neutropenia, anemia, and/or thrombocytopenia. This condition is often associated with autoimmune disorders, especially rheumatoid arthritis, and other lymphoproliferative disorders. The diagnosis is suggested by flow cytometry demonstrating an expansion of CD8⁺CD57⁺ T cells and is confirmed by T-cell receptor gene rearrangement studies. Mounting evidence suggests that LGL leukemia is a disorder of dysregulation of apoptosis through abnormalities in the Fas/Fas ligand pathway. In most patients, this is an indolent disorder, and significant improvement of cytopenias can be achieved with immunosuppressive agents such as steroids, methotrexate, cyclophosphamide, and cyclosporin A. This review provides a concise, up-to-date summary of LGL leukemia and the related, more aggressive, malignancies of cytotoxic T cells and natural killer cells.

Key Words. Chronic T-cell leukemia • Felty’s syndrome • Neutropenia • Natural killer cell

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