title: Acquired pure red cell aplasia associated with lymphoproliferative disease of granular T lymphocytes.
abstract: Acquired pure red cell aplasia (PRCA) can be associated with lymphoproliferative disease of granular T lymphocytes (T-LDGL), also known as T-cell large granular lymphocyte leukemia. Fifteen adult patients with PRCA associated with T-LDGL comprise this study. Neutropenia and rheumatoid arthritis were uncommon. All patients responded to immunosuppressive therapy. The 2 most commonly used treatments were prednisone and cyclophosphamide +/- corticosteroids, producing overall response rates of 50% and 60%, respectively. Treatment with cyclophosphamide was associated with a more durable remission (median, 60 versus 7.5 months). After a median follow-up of 67 months, 2 patients died of treatment-related complications, one from myelodysplasia and another from cyclosporine-induced renal failure. The clinical course and treatment responses of PRCA associated with T-LDGL in this series were similar to the general group of PRCA. Because T-LDGL is frequently underdiagnosed, it is likely that a significant proportion of idiopathic or primary PRCA is in fact secondary to T-LDGL.
authors: Divisions of Hematology and Hematopathology, Mayo Clinic and Foundation, Rochester, MN 55905, USA.
authors: R S
authors: RS
authors: Go
authors: C Y
authors: CY
authors: Li
authors: A
authors: A
authors: Tefferi
authors: R L
authors: RL
authors: Phyliky
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001172:Arthritis, Rheumatoid / Q000150:complications; D003520:Cyclophosphamide / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015458:Leukemia, T-Cell / Q000150:complications* / Q000188:drug therapy; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D009503:Neutropenia / Q000150:complications; D011241:Prednisone / Q000627:therapeutic use; D012008:Recurrence; D012010:Red-Cell Aplasia, Pure / Q000150:complications* / Q000188:drug therapy; D012074:Remission Induction; D013602:T-Lymphocytes, Cytotoxic / Q000473:pathology
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D003520:Cyclophosphamide; D011241:Prednisone; D008727:Methotrexate