MRI multicentre prospective survey in thalassaemia major patients treated with deferasirox versus deferiprone and desferrioxamine

Br J Haematol. 2018 Dec;183(5):783-795. doi: 10.1111/bjh.15595. Epub 2018 Oct 18.

Abstract

We prospectively assessed the efficacy of deferasirox versus deferiprone or desferrioxamine as monotherapy in thalassaemia major (TM) patients by magnetic resonance imaging (MRI). We selected the patients enrolled in the Myocardial Iron Overload in Thalassaemia network who received only one chelator between two MRIs (deferasirox = 235, deferiprone = 142, desferrioxamine = 162). Iron overload was measured by T2* technique and biventricular function by cine images. Among the patients with baseline myocardial iron, in all three groups there was a significant improvement in global heart T2* values. The deferiprone and desferrioxamine groups showed a significant improvement in left ventricular ejection fraction (LVEF). Only the deferiprone group showed a significant improvement in right ventricular ejection fraction (RVEF). The improvement in global heart T2* was significantly lower in the deferasirox versus the deferiprone group. The improvement in the LVEF was significantly higher in the deferiprone and desferrioxamine groups than in the deferasirox group and the improvement in the RVEF was significantly higher in the deferiprone than in deferasirox group. Among the patients with baseline hepatic iron, the changes in hepatic iron were comparable in deferasirox versus the other groups. Deferasirox monotherapy was less effective than deferiprone in improving myocardial siderosis and biventricular function and less effective than desferrioxamine in improving the LVEF.

Keywords: chelation therapy; iron overload; magnetic resonance imaging; thalassaemia major.

Publication types

  • Comparative Study
  • Multicenter Study
  • Observational Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Cardiomyopathies / complications
  • Cardiomyopathies / drug therapy
  • Deferasirox / therapeutic use*
  • Deferiprone / therapeutic use*
  • Deferoxamine / therapeutic use*
  • Drug Substitution
  • Drug Therapy, Combination
  • Female
  • Humans
  • Iron Chelating Agents / therapeutic use*
  • Iron Overload / complications
  • Iron Overload / drug therapy
  • Magnetic Resonance Imaging
  • Male
  • Prospective Studies
  • Treatment Outcome
  • beta-Thalassemia / complications
  • beta-Thalassemia / drug therapy*

Substances

  • Iron Chelating Agents
  • Deferiprone
  • Deferoxamine
  • Deferasirox