title: Post-transfusional iron overload in the haemoglobinopathies.
abstract: In this report, we review the recent advances in evaluation and treatment of transfusional iron overload (IO). Results of the French thalassaemia registry are described. According to the disease, thalassaemia major or sickle cell anaemia, mechanisms and toxicity of iron overload, knowledge about IO long-term outcome and chelation treatment results, respective value of IO markers, differ. The recent tools evaluating organ specific IO and the diversification of iron chelator agents make possible to individualize chelation therapy in clinical practice. The severity of IO and the level of transfusional iron intake, the preferential localization of IO (heart/liver) as well as the tolerance and adherence profiles of the patient can now be taken into account. Introduction of cardiac magnetic resonance imaging for the quantification of myocardial iron and use of oral chelators have already been reported as decreasing the cardiac mortality rate related to IO in thalassaemia major patients. Long-term observation of patients under oral chelators will show if morbidity is also improving via a more continuous control of toxic iron and/or a better accessibility to cellular iron pools.
authors: Service d'hématologie pédiatrique, centre de référence des Thalassémies, hôpital d'Enfants de la Timone, AP-HM, 13385 Marseille cedex 5, France. isabelle.thuret@ap-hm.fr
authors: Isabelle
authors: I
authors: Thuret
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications / Q000628:therapy*; D015913:Chelation Therapy; D002648:Child; D017322:Clinical Trials, Phase II as Topic; D004359:Drug Therapy, Combination; D005293:Ferritins / Q000097:blood; D005602:France / Q000453:epidemiology; D006801:Humans; D007501:Iron / Q000032:analysis / Q000493:pharmacokinetics; D007502:Iron Chelating Agents / Q000009:adverse effects / Q000627:therapeutic use; D019190:Iron Overload / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology / Q000517:prevention & control; D008099:Liver / Q000737:chemistry; D008279:Magnetic Resonance Imaging; D055118:Medication Adherence; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D012042:Registries; D013789:Thalassemia / Q000097:blood / Q000150:complications / Q000453:epidemiology / Q000628:therapy*; D065227:Transfusion Reaction
chemical_list: D007502:Iron Chelating Agents; D005293:Ferritins; D007501:Iron