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Long-term treatment with deferiprone enhances left ventricular ejection function when compared to deferoxamine in patients with thalassemia major.

Abstract
Transfusion and iron chelation treatment have significantly reduced morbidity and improved survival of patients with thalassemia major. However, cardiac disease continues to be the most common cause of death. We report the left-ventricular ejection fraction, determined by echocardiography, in one hundred sixty-eight patients with thalassemia major followed for at least 5years who received continuous monotherapy with deferoxamine (N=108) or deferiprone (N=60). The statistical analysis, using the generalized estimating equations model, indicated that the group treated with deferiprone had a significantly better left-ventricular ejection fraction than did those treated with deferoxamine (coefficient 0.97; 95% CI 0.37; 1.6, p=0.002). The heart may be particularly sensitive to iron-induced mitochondrial damage because of the large number of mitochondria and its low level of antioxidants. Deferiprone, because of its lower molecular weight, might cross into heart mitochondria more efficiently, improving their activity and, thereby, myocardial cell function. Our findings indicate that the long-term administration of deferiprone significantly enhances left-ventricular function over time in comparison with deferoxamine treatment. However, because of limitations related to the design of this study, these findings should be confirmed in a prospective, randomized clinical trial.
AuthorsAldo Filosa, Angela Vitrano, Paolo Rigano, Giuseppina Calvaruso, Rita Barone, Marcello Capra, Liana Cuccia, Francesco Gagliardotto, Lorella Pitrolo, Luciano Prossomariti, Maddalena Casale, Vincenzo Caruso, Calogera Gerardi, Saveria Campisi, Paolo Cianciulli, Michele Rizzo, Giuseppe D'Ascola, Angela Ciancio, Aurelio Maggio
JournalBlood cells, molecules & diseases (Blood Cells Mol Dis) Vol. 51 Issue 2 Pg. 85-8 (Aug 2013) ISSN: 1096-0961 [Electronic] United States
PMID23628348 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
CopyrightCopyright © 2013 Elsevier Inc. All rights reserved.
Chemical References
  • Iron Chelating Agents
  • Pyridones
  • Deferiprone
  • Deferoxamine
Topics
  • Adult
  • Deferiprone
  • Deferoxamine (therapeutic use)
  • Female
  • Heart Diseases (drug therapy, etiology, physiopathology)
  • Humans
  • Iron Chelating Agents (therapeutic use)
  • Iron Overload (drug therapy, etiology)
  • Male
  • Pyridones (therapeutic use)
  • Retrospective Studies
  • Stroke Volume (drug effects)
  • Treatment Outcome
  • Ventricular Function, Left (drug effects)
  • Young Adult
  • beta-Thalassemia (complications)

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