Severe congenital dyserythropoietic anaemia type I: prenatal management, transfusion support and alpha‐interferon therapy

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Abstract
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, α‐interferon therapy was initiated (106 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 α‐interferon was decreased to 106 units twice a week. No relapse of anaemia was noted during an additional 4 months of follow‐up.