Diamond-Blackfan anemia: clinical features and treatment results in 4 cases.

Diamond-Blackfan anemia is a rare hematological disease characterized by selective marrow erythroid hypoplasia. We present the clinical and hematological profile and results of treatment in four children. The median age at presentation was 14 months with a median age of onset of pallor at 7.5 months. Mean Hb at presentation was 2.9 g/dl. All children were started on prednisolone with response in one child. One child was switched to dexamethasone and responded transiently relapsing in 4 months but responded to further dexamethasone. Two children required cyclosporin for 3 and 8 weeks for response. With median follow-up of 59 months, all four children continue in complete hematological remission with no further relapses.