Clinico-haematological profile of pure red cell aplasia in children.

Abstract

Pure red cell aplasia (PRCA) is a rare disorder, characterized by isolated failure of erythropoiesis. The clinico-haematological profile of 16 patients with PRCA is presented in this communication. Fourteen patients had Diamond-Blackfan anaemia (DBA), one had transient erythroblastopenia of childhood, and one patient had PRCA secondary to carbamazepine. Physical abnormalities were observed in 50 per cent of patients with DBA. Of the nine patients with DBA who were administered prednisolone and had a regular follow-up, four (44.4 per cent) had no response, three (33.3 per cent) responded fully, and two (22.2 per cent) were steroid dependent.