title: Hydroxyurea can eliminate transfusion requirements in children with severe beta-thalassemia.
abstract: Hydroxyurea (HU) enhances fetal hemoglobin (Hb) production. An increase in total Hb level has been repeatedly reported during HU treatment in patients with sickle cell disease and in several patients with beta-thalassemia intermedia. Effects in patients with beta-thalassemia major are controversial. We now report a marked elevation of total Hb levels with HU that permitted regular transfusions to be stopped in 7 children with transfusion-dependent beta-thalassemia. The median follow-up was 19 +/- 3 months (range, 13-21 months). We conclude that HU can eliminate transfusional needs in children with beta-thalassemia major, which could be particularly useful in countries such as Algeria, where supplies of blood or chelating agents are limited.
authors.affiliation: Service d'Hématologie, Hôpital Franz Fanon, Blida, Algeria.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Bradai
authors.forename: Mohand Tayeb
authors.initials: MT
authors.lastname: Abad
authors.forename: Serge
authors.initials: S
authors.lastname: Pissard
authors.forename: Fatima
authors.initials: F
authors.lastname: Lamraoui
authors.forename: Laurent
authors.initials: L
authors.lastname: Skopinski
authors.forename: Mariane
authors.initials: M
authors.lastname: de Montalembert
mesh_terms: D000293:Adolescent; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006454:Hemoglobins / Q000032:analysis / Q000096:biosynthesis / Q000187:drug effects; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D008297:Male; D016896:Treatment Outcome; D017086:beta-Thalassemia / Q000097:blood / Q000188:drug therapy* / Q000628:therapy
chemical_list: D006454:Hemoglobins; D006918:Hydroxyurea