title: Successful Treatment of Iron-Overload Cardiomyopathy in Hereditary Hemochromatosis With Deferoxamine and Deferiprone.
abstract: There is scarce evidence regarding the use of iron chelators in patients with hereditary hemochromatosis who are intolerant of phlebotomy or erythrocytapheresis. A 52-year-old man with genetically confirmed HFE hemochromatosis presented with liver disease and heart failure with severe left ventricular systolic dysfunction. Because of anemia after initial treatment, we added intravenous deferoxamine followed by oral deferiprone to less frequent erythrocytapheresis, which normalized systolic function within 1 year. Repeated cardiac magnetic resonance imaging revealed improvement of the T2* relaxation time. This report illustrates the beneficial effect of iron chelators in individuals with HFE hemochromatosis and poor tolerance of erythrocytapheresis.
authors: Department of Cardiology, IKEM, Prague, Czech Republic.
authors: Lydie
authors: L
authors: Tauchenová
authors: Department of Cardiology, IKEM, Prague, Czech Republic.
authors: Barbora
authors: B
authors: Křížová
authors: Department of Cardiology, IKEM, Prague, Czech Republic. Electronic address: mikb@ikem.cz.
authors: Miloš
authors: M
authors: Kubánek
authors: Department of Hepatogastroenterology, IKEM, Prague, Czech Republic.
authors: Soňa
authors: S
authors: Fraňková
authors: Department of Cardiology, IKEM, Prague, Czech Republic.
authors: Vojtěch
authors: V
authors: Melenovský
authors: Department of Radiology, IKEM, Prague, Czech Republic.
authors: Jaroslav
authors: J
authors: Tintěra
authors: Department of Radiology, IKEM, Prague, Czech Republic.
authors: Dana
authors: D
authors: Kautznerová
authors: Department of Pathology, IKEM, Prague, Czech Republic.
authors: Jana
authors: J
authors: Malušková
authors: Department of Pathology, IKEM, Prague, Czech Republic; Department of Experimental Medicine, IKEM, Prague, Czech Republic.
authors: Milan
authors: M
authors: Jirsa
authors: Department of Cardiology, IKEM, Prague, Czech Republic.
authors: Josef
authors: J
authors: Kautzner
mesh_terms: D009202:Cardiomyopathies / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D000077543:Deferiprone; D003676:Deferoxamine / Q000008:administration & dosage*; D005293:Ferritins / Q000032:analysis; D006333:Heart Failure / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D006432:Hemochromatosis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D000071020:Hemochromatosis Protein / Q000235:genetics; D006801:Humans; D007502:Iron Chelating Agents / Q000008:administration & dosage; D019190:Iron Overload / Q000097:blood / Q000150:complications; D008107:Liver Diseases / Q000175:diagnosis / Q000209:etiology; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods; D008297:Male; D008875:Middle Aged; D011728:Pyridones / Q000008:administration & dosage*; D012720:Severity of Illness Index; D013318:Stroke Volume; D014168:Transferrin / Q000032:analysis; D016896:Treatment Outcome; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis / Q000209:etiology
chemical_list: C103673:HFE protein, human; D000071020:Hemochromatosis Protein; D007502:Iron Chelating Agents; D011728:Pyridones; D014168:Transferrin; D000077543:Deferiprone; D005293:Ferritins; D003676:Deferoxamine