Abstract • 98
A severe aregenerative anemia (Hb : 3.5g/dL) detected in a female fetus resulted in 2 antenatal exchange transfusions at 28 and 31 weeks gestational age (GA). The newborn delivered at 35 weeks GA had to be transfused at birth and entered thereafter a regular transfusion program. Congenital dyserythropoietic anemia (CDA) type I was confirmed by electron microscopy "swiss cheese" typical abnormality of the heterochromatin. At 14 months of age, after 15 post-natal red blood cell transfusions, serum ferritin was 1300ng/mL. Interferon α therapy (IFNα) (subcutaneous; 3M U/week) resulted in a sustained rise in reticulocyte and in Hb values ≥ 11 g/dL, allowing transfusion independence (follow-up : 4 months). IFNα beneficial effect in CDA type I has been reported after the fortuitous observation of a patient who presented with both the disease and chronic hepatitis C. The mechanisms which lead IFNα to restore erythropoiesis remain a matter of speculation.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Tchernia, G., Dommergues, M., Zupan, V. et al. Severe congenital dyserythropoietic anemia type I. Antenatal management, transfusion support, and Interferon α therapy. Pediatr Res 45 (Suppl 5), 759 (1999). https://doi.org/10.1203/00006450-199905010-00128
Issue Date:
DOI: https://doi.org/10.1203/00006450-199905010-00128