Acquired pure red cell aplasia associated with lymphoproliferative disease of granular T lymphocytes

Blood. 2001 Jul 15;98(2):483-5. doi: 10.1182/blood.v98.2.483.


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.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Aged
  • Aged, 80 and over
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Arthritis, Rheumatoid / complications
  • Cyclophosphamide / therapeutic use
  • Cyclosporine / therapeutic use
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Leukemia, T-Cell / complications*
  • Leukemia, T-Cell / drug therapy
  • Male
  • Methotrexate / therapeutic use
  • Middle Aged
  • Neutropenia / complications
  • Prednisone / therapeutic use
  • Recurrence
  • Red-Cell Aplasia, Pure / complications*
  • Red-Cell Aplasia, Pure / drug therapy
  • Remission Induction
  • T-Lymphocytes, Cytotoxic / pathology


  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Cyclosporine
  • Cyclophosphamide
  • Prednisone
  • Methotrexate