title: Transfusional hemosiderosis and combined chelation therapy in sickle thalassemia.
abstract: Although the indications for transfusions in sickle cell syndromes are well listed, and chronic transfusion has become practicable since the recent advances in chelation therapy have essentially eliminated the risk of secondary iron overload, multi-transfused, non-compliant to long-term chelation therapy patients confront the complication of iron overload and secondary hemosiderosis. In thalassemia major patients, combined therapy with desferrioxamine and deferiprone has maximized tissue iron removal and may reduce the overall occurrence of hemosiderotic heart failure. Despite this, safety and contradictions of chelating agents are still controversial. The aim of this report is to present the results of this combination in a long-term transfused sickle beta-thalassemic patient suffering from severe heart failure and liver dysfunction.
authors.affiliation: First Department of Internal Medicine, University of Athens, Medical School, Laiko Hospital, Athens, Greece. gpoyl@otenet.gr
authors.forename: M
authors.initials: M
authors.lastname: Tsironi
authors.forename: K
authors.initials: K
authors.lastname: Polonifi
authors.forename: S
authors.initials: S
authors.lastname: Deftereos
authors.forename: D
authors.initials: D
authors.lastname: Farmakis
authors.forename: P
authors.initials: P
authors.lastname: Andriopoulos
authors.forename: I
authors.initials: I
authors.lastname: Moyssakis
authors.forename: A
authors.initials: A
authors.lastname: Aessopos
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D001803:Blood Transfusion; D015913:Chelation Therapy; D000077543:Deferiprone; D003676:Deferoxamine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D006333:Heart Failure; D006486:Hemosiderosis / Q000209:etiology / Q000517:prevention & control*; D006801:Humans; D017093:Liver Failure; D008297:Male; D011728:Pyridones / Q000627:therapeutic use; D017086:beta-Thalassemia / Q000150:complications / Q000188:drug therapy / Q000628:therapy*
chemical_list: D011728:Pyridones; D000077543:Deferiprone; D003676:Deferoxamine