Successful Treatment of Iron-Overload Cardiomyopathy in Hereditary Hemochromatosis With Deferoxamine and Deferiprone

Can J Cardiol. 2016 Dec;32(12):1574.e1-1574.e3. doi: 10.1016/j.cjca.2016.07.589. Epub 2016 Jul 28.

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.

Publication types

  • Case Reports

MeSH terms

  • Cardiomyopathies* / diagnosis
  • Cardiomyopathies* / etiology
  • Cardiomyopathies* / physiopathology
  • Cardiomyopathies* / therapy
  • Deferiprone
  • Deferoxamine / administration & dosage*
  • Ferritins / analysis
  • Heart Failure* / diagnosis
  • Heart Failure* / drug therapy
  • Heart Failure* / etiology
  • Hemochromatosis Protein / genetics
  • Hemochromatosis* / blood
  • Hemochromatosis* / diagnosis
  • Hemochromatosis* / drug therapy
  • Hemochromatosis* / physiopathology
  • Humans
  • Iron Chelating Agents / administration & dosage
  • Iron Overload / blood
  • Iron Overload / complications
  • Liver Diseases / diagnosis
  • Liver Diseases / etiology
  • Magnetic Resonance Imaging, Cine / methods
  • Male
  • Middle Aged
  • Pyridones / administration & dosage*
  • Severity of Illness Index
  • Stroke Volume
  • Transferrin / analysis
  • Treatment Outcome
  • Ventricular Dysfunction, Left / diagnosis
  • Ventricular Dysfunction, Left / etiology

Substances

  • HFE protein, human
  • Hemochromatosis Protein
  • Iron Chelating Agents
  • Pyridones
  • Transferrin
  • Deferiprone
  • Ferritins
  • Deferoxamine