1. Severe congenital dyserythropoietic anaemia type I: prenatal management, transfusion support and alpha-interferon therapy
- Author
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Sunitha N. Wickramasinghe, Jean-Paul Dommergues, V. Zupan, Jacques Delaunay, M. Dommergues, F Mielot, Gil Tchernia, N. Parez, Em Cramer, H. Chambost, and J. B. Fieschi
- Subjects
Adult ,medicine.medical_specialty ,Pediatrics ,Iron Overload ,Pregnancy Trimester, Third ,medicine.medical_treatment ,Exchange Transfusion, Whole Blood ,Alpha interferon ,Interferon alpha-2 ,Congenital dyserythropoietic anemia type I ,Liver Function Tests ,Pregnancy ,Prenatal Diagnosis ,Humans ,Medicine ,Caesarean section ,Interferon alfa ,Anemia, Dyserythropoietic, Congenital ,Fetus ,business.industry ,Infant, Newborn ,Infant ,Interferon-alpha ,Bone Marrow Examination ,Hematology ,medicine.disease ,Recombinant Proteins ,Surgery ,Treatment Outcome ,Pregnancy Trimester, Second ,Gestation ,Female ,business ,Congenital dyserythropoietic anemia ,Dyserythropoietic anemia ,medicine.drug - 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, alpha-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 alpha-interferon was decreased to 106 units twice a week. No relapse of anaemia was noted during an additional 4 months of follow-up.
- Published
- 2000
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