title: Iron chelation therapy in the management of transfusion-related cardiac iron overload.
abstract: Iron overload is one of the major causes of morbidity and death in patients undergoing chronic transfusion therapy. Furthermore, excessive iron accumulation in the heart may result in impaired left ventricular dysfunction. With accurate monitoring techniques and treatment regimens, progression of heart complications can be followed, and their natural history changed. Iron chelation therapy is the mainstay of prevention and reversal of myocardial iron overload. Despite recent appraisals of general chelating strategies, the management of iron chelation in chronically transfused patients with a focus on the heart has not been extensively assessed. New studies published in the past couple of years have provided important new data in this topic and therefore this review summarizes the major studies that examined the removal of iron from the heart with the iron chelators: deferoxamine, deferiprone, and deferasirox. Since chronically transfused patients and their cardiac clinical presentations vary widely, this review tries to identify--with each drug--the precise scenarios evaluated, linking patients' baseline characteristics, clinical setting, and drug intake and dosing. Ultimately, by stratifying patients according to their cardiac iron overload status and ventricular function, this review identifies possible approaches for the initial treatment and follow-up of transfusion-related cardiac iron overload.
authors.affiliation: University of Campinas, São Paulo, Brazil. jlaraf@fcm.unicamp.br
authors.forename: Juliano Lara
authors.initials: JL
authors.lastname: Fernandes
mesh_terms: D001565:Benzoates / Q000008:administration & dosage / Q000627:therapeutic use; D002311:Cardiomyopathy, Dilated / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D015913:Chelation Therapy; D003430:Cross-Sectional Studies; D000077588:Deferasirox; D000077543:Deferiprone; D003676:Deferoxamine / Q000008:administration & dosage / Q000627:therapeutic use; D004311:Double-Blind Method; D004359:Drug Therapy, Combination; D005544:Forecasting; D006801:Humans; D007502:Iron Chelating Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D019190:Iron Overload / Q000188:drug therapy* / Q000209:etiology; D008279:Magnetic Resonance Imaging; D017410:Practice Guidelines as Topic; D011446:Prospective Studies; D011728:Pyridones / Q000008:administration & dosage / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic; D012189:Retrospective Studies; D065227:Transfusion Reaction; D016896:Treatment Outcome; D014230:Triazoles / Q000008:administration & dosage / Q000627:therapeutic use; D018487:Ventricular Dysfunction, Left / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology
chemical_list: D001565:Benzoates; D007502:Iron Chelating Agents; D011728:Pyridones; D014230:Triazoles; D000077543:Deferiprone; D003676:Deferoxamine; D000077588:Deferasirox