Severe congenital dyserythropoietic anaemia type I: prenatal management, transfusion support and alpha-interferon therapy
| Autor: | Sunitha N. Wickramasinghe, Jean-Paul Dommergues, V. Zupan, Jacques Delaunay, M. Dommergues, F Mielot, Gil Tchernia, N. Parez, Em Cramer, H. Chambost, J. B. Fieschi |
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| Rok vydání: | 2000 |
| Předmět: |
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 |
| Zdroj: | British Journal of Haematology. 110:420-423 |
| ISSN: | 0007-1048 |
| DOI: | 10.1046/j.1365-2141.2000.02168.x |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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