Lymphoproliferative disease of granular T lymphocytes presenting as aplastic anemia.
Lymphoproliferative disease of granular T lymphocyte (T-LDGL), also known as T-cell large granular lymphocyte leukemia, is a clonal disorder of cytotoxic T lymphocytes that is clinically manifested as chronic neutropenia and anemia. Association with autoimmune disorders is common. In 9 patients, T-LDGL is reported as presenting as aplastic anemia. The clinical characteristics were similar to acquired aplastic anemia. Morphologic evidence of increased granular lymphocytes in the peripheral blood and an excess of CD3(+)/CD8(+)/CD57(+) cells in the bone marrow were found in most cases. Cyclophosphamide was ineffective, but noncytotoxic immunosuppressive agents generally produced a good response. After a median follow-up of 49 months, 5 patients had died from the disease or related complications. Median survival was 40 months. Aplastic anemia can be a presenting manifestation of T-LDGL, and T-LDGL should be considered in the differential diagnosis of acquired aplastic anemia.
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Divisions of Hematology and Hematopathology, Mayo Clinic and Foundation, Rochester, MN, USA.
Tefferi
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Lust
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Phyliky
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D000328:Adult; D000368:Aged; D000741:Anemia, Aplastic / Q000188:drug therapy / Q000209:etiology*; D001854:Bone Marrow Cells / Q000276:immunology; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000592:standards; D020732:Cytogenetic Analysis; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016130:Immunophenotyping; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000592:standards; D007945:Leukemia, Lymphoid / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D015458:Leukemia, T-Cell / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage / Q000592:standards; D015996:Survival Rate; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone