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Blood, 15 July 2001, Vol. 98, No. 2, pp. 483-485
BRIEF REPORT
Acquired pure red cell aplasia associated with
lymphoproliferative disease of granular T lymphocytes
Ronald S. Go,
Chin-Yang Li,
Ayalew Tefferi, and
Robert L. Phyliky
From the Divisions of Hematology and Hematopathology,
Mayo Clinic and Foundation, Rochester, MN.
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.
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