Effect of combined chelation therapy with deferiprone and deferoxamine on left ventricular diastolic function in adult beta-thalassemia major patients

Hemoglobin. 2010 Jun;34(3):210-20. doi: 10.3109/03630269.2010.485120.

Abstract

Beta-thalassemia major (beta-TM), patients, asymptomatic and with preserved left ventricular ejection fraction (LVEF) were studied echocardiographically. Group A (26 patients), on deferiprone (L1) and deferoxamine (DFO) combination therapy (L1: 80 +/- 27 mg/kg/day, DFO: 160 +/- 87 mg/kg/week) and group B (35 patients) on DFO monotherapy (240 +/- 40 mg/kg/week) for the last 2 years were compared. Another group, C (14 patients), switched to L1 (74 +/- 15 mg/kg/day) plus DFO (158 +/- 48 mg/kg/week) for 20-30 months, was prospectively studied for 2 years. In group A, MRI T2* values were increased and improved in group C during follow-up. The LVEF was better in group A than in group B, while such an improvement was also detected in the group C follow-up study. The Tissue Doppler study E' velocity and E/E' ratio was not different. Similarly, in the group C follow-up no significant change in E/E' ratio was detected. It seems that although LVEF significantly improves with combined therapy, diastolic function indexes do not show a similar change.

MeSH terms

  • Adult
  • Chelation Therapy / methods*
  • Deferiprone
  • Deferoxamine / administration & dosage
  • Deferoxamine / therapeutic use
  • Drug Therapy, Combination
  • Echocardiography
  • Female
  • Follow-Up Studies
  • Humans
  • Iron / metabolism
  • Iron Chelating Agents / administration & dosage
  • Iron Chelating Agents / therapeutic use*
  • Male
  • Prospective Studies
  • Pyridones / administration & dosage
  • Pyridones / therapeutic use*
  • Ventricular Dysfunction, Left / complications
  • Ventricular Dysfunction, Left / drug therapy*
  • Ventricular Dysfunction, Left / physiopathology*
  • beta-Thalassemia / complications
  • beta-Thalassemia / drug therapy*

Substances

  • Iron Chelating Agents
  • Pyridones
  • Deferiprone
  • Iron
  • Deferoxamine