Lymphoproliferative disease of granular T lymphocytes presenting as aplastic anemia

Lymphoproliferative disease of granular T lymphocyte (T-LDGL), also known a s T-cell large granular lymphocyte leukemia, is a clonal disorder of cytoto xic T lymphocytes that is clinically manifested as chronic neutropenia and anemia. Association with autoimmune disorders is common, in 9 patients, T-L DGL is reported as presenting as aplastic anemia. The clinical characterist ics were similar to acquired aplastic anemia. Morphologic evidence of incre ased granular lymphocytes in the peripheral blood and an excess of CD3(+)/C D8(+)/CD57(+) cells in the bone marrow were found in most cases. Cyclophosp hamide was ineffective, but noncytotoxic immunosuppressive agents generally produced a good response. After a median follow-up of 49 months, 5 patient s had died from the disease or related complications. Median survival was 4 0 months. Aplastic anemia can be a presenting manifestation of T-LDGL, and T-LDGL should be considered in the differential diagnosis of acquired aplas tic anemia. (C) 2000 by The American Society of Hematology.