Abstract
Pure red cell aplaisa (PRCA) is a rare, refractory atuo-immune disease, and produces chronic progressive anemia because of a failure of erythropoiesis. Half the patients with PRCA are associated with thymoma. Thymomectomy or thymectomy is not sufficient to restore the hemopoiesis in this disease. Steroid therapy is thought to be the most useful treatment for PRCA, but the effective dosage of steroids is controversial. We reviewed cases of PRCA from the viewpoint of sterioid therapy, including these 2 cases (one died of post-trasfusion hepatitis, and the another died of penumonia due to massive steroid dosage). As a result, conventional steroid dosage (<0.5mg/kg/day) may not be sufficient to improve anemia, but massive dosages (1mgkg/day-1, 5mg/kg/day) seem to be optimal for restoring the hemopoiesis.
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