Regression of myocardial dysfunction after switching from desferrioxamine to deferiprone therapy in beta-thalassemia major patients

Hemoglobin. 2006;30(2):229-38. doi: 10.1080/03630260600642559.

Abstract

This study tries to compare the cardioprotective effects of the desferrioxamine (DFO) and deferiprone (L1) therapies on thalassemia major patients. We enrolled nine patients with thalassemia major complicated by some degree of myocardial dysfunction. These patients, recipients of >10 years of DFO injection therapy, were switched from DFO to L1. Echocardiographic measures of left ventricular systolic, diastolic and global functions, were assessed regularly every 6 months. Mean values of each parameter for the DFO and L1 treatment periods were compared using paired t-test and Wilcoxon signed-rank test. Global cardiac function improved significantly. Myocardial dysfunction in patients with thalassemia major can be reversed by regular use of the oral iron chelator L1.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Cardiomegaly / drug therapy
  • Cardiomegaly / etiology
  • Cardiomyopathies / drug therapy*
  • Cardiomyopathies / etiology
  • Chelation Therapy*
  • Cohort Studies
  • Combined Modality Therapy
  • Deferiprone
  • Deferoxamine / therapeutic use*
  • Drug Evaluation
  • Exercise Tolerance / drug effects
  • Female
  • Follow-Up Studies
  • Heart Failure / drug therapy
  • Heart Failure / etiology
  • Hemosiderosis / drug therapy*
  • Hemosiderosis / etiology
  • Humans
  • Infant
  • Iron Chelating Agents / therapeutic use*
  • Male
  • Pyridones / therapeutic use*
  • Retrospective Studies
  • Stroke Volume / drug effects
  • Transfusion Reaction
  • beta-Thalassemia / complications
  • beta-Thalassemia / drug therapy*
  • beta-Thalassemia / therapy

Substances

  • Iron Chelating Agents
  • Pyridones
  • Deferiprone
  • Deferoxamine