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.
Résumé
Il existe peu de données probantes au sujet de l’utilisation des chélateurs du fer
chez les patients atteints d’une hémochromatose héréditaire qui ne tolèrent pas la
phlébotomie ou l’érythrocytaphérèse. Un homme de 52 ans souffrant d’une hémochromatose
génétique de type HFE confirmée a présenté une maladie du foie et une insuffisance
cardiaque associée à une dysfonction systolique importante du ventricule gauche. En
raison de l’anémie après le traitement initial, nous avons ajouté la déféroxamine
par voie intraveineuse, suivie de la défériprone par voie orale, et diminué la fréquence
des érythrocytaphérèses, ce qui a normalisé la fonction systolique en 1 année. L’imagerie
cardiaque répétée par résonance magnétique a révélé l’amélioration du temps de relaxation
T2*. Le présent rapport illustre les effets bénéfiques des chélateurs du fer chez
les individus atteints d’une hémochromatose HFE qui tolèrent mal l’érythrocytaphérèse.
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References
- Hereditary hemochromatosis—a new look at an old disease.N Engl J Med. 2004; 350: 2383-2397
- Iron-overload cardiomyopathy: pathophysiology, diagnosis, and treatment.J Card Fail. 2010; 16: 888-900
- End-stage cardiomyopathy because of hereditary hemochromatosis successfully treated with erythrocytapheresis in combination with left ventricular assist device support.Circ Heart Fail. 2014; 7: 541-543
- Reversal of cardiac complications by deferiprone and deferoxamine combination therapy in a patient affected by a severe type of juvenile hemochromatosis.Blood. 2007; 109: 362-364
- Deferasirox in patients with iron overload secondary to hereditary hemochromatosis: results of a 1-yr phase 2 study.Eur J Haematol. 2015; 95: 545-550
Article info
Publication history
Published online: July 29, 2016
Accepted:
July 25,
2016
Received:
June 22,
2016
Footnotes
See page 1574.e3 for disclosure information.
Identification
Copyright
© 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
