Summary. We report a case of congenital dyserythropoietic anaemia, type I, with severe pre-and postnatal manifestations. Exchange transfusions were required for fetal anaemia (3´5 g/dl) at 28 and 30 weeks of gestation. Transfusions were administered at birth (Caesarean section at week 35) and at regular intervals thereafter. At 14 months, a-interferon therapy was initiated (10 6 units three times a week). This resulted in stabilization of the haemoglobin at or above 11 g/dl and a reduction in the percentage of erythroblasts with ultrastructurally abnormal heterochromatin. After 9 months, the dose of a-interferon was decreased to 10 6 units twice a week. No relapse of anaemia was noted during an additional 4 months of follow-up.

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