Hydroxyurea in Congenital Erythropoietic Porphyria
- 14 April 1994
- journal article
- letter
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 330 (15) , 1091-1092
- https://doi.org/10.1056/nejm199404143301519
Abstract
Congenital erythropoietic porphyria is a defect of uroporphyrinogen III synthase, with accumulation of uroporphyrin I, extreme hemolysis, and photosensitivity1,2. We previously reported the effectiveness of aggressive transfusion in a patient with severe disease3. The excess porphyrins were mostly of erythropoietic origin.Keywords
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