title: Effects of chelation therapy on cardiac function improvement in thalassemia patients: literature review and the Taiwanese experience.
abstract: The iron chelators deferoxamine (DFO) and deferiprone (L1) have demonstrated their ability to normalize cardiac function in patients with iron overload-induced cardiac disease. However, conventional chelation with subcutaneous DFO fails to prevent iron deposition in two-thirds of thalassemia major patients, placing them at risk of heart failure and its complications. Deferiprone appears to be more effective in cardiac iron removal. The detection and management of heart complications have improved dramatically over the last 7 years. Non invasive techniques of quantifying iron burden via magnetic resonance imaging (MRI) have been validated. A better understanding of cardiac pathophysiology and improved ability to detect at-risk populations are yielding better outcomes and reduced morbidity. We continue to appraise readily available bedside tools for monitoring thalassemia patients with heart complications, and here we summarize studies from the literature and our own findings. Deferiprone chelation was found to be of statistically significant benefit in upgrading cardiac function and reducing iron accumulation. The use of echocardiography and MRI to closely monitor cardiac functions associated with iron overload complications and mortality has proved quite practical.
authors.affiliation: Department of Laboratory Medicine, China Medical University & Hospital, Taichung, Taiwan. pct@mail.cmuh.org.tw
authors.forename: Ching-Tien
authors.initials: CT
authors.lastname: Peng
authors.forename: Chang-Hai
authors.initials: CH
authors.lastname: Tsai
authors.forename: Kang-Hsi
authors.initials: KH
authors.lastname: Wu
mesh_terms: D009202:Cardiomyopathies / Q000188:drug therapy* / Q000209:etiology / Q000378:metabolism; D015913:Chelation Therapy; D000077543:Deferiprone; D003676:Deferoxamine / Q000008:administration & dosage / Q000627:therapeutic use*; D005293:Ferritins / Q000097:blood; D006801:Humans; D007501:Iron / Q000378:metabolism; D007502:Iron Chelating Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D019190:Iron Overload / Q000188:drug therapy* / Q000209:etiology; D009206:Myocardium / Q000378:metabolism; D011728:Pyridones / Q000008:administration & dosage / Q000627:therapeutic use*; D013624:Taiwan; D013789:Thalassemia / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology
chemical_list: D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D005293:Ferritins; D007501:Iron; D003676:Deferoxamine