Effects of Chelation Therapy on Cardiac Function Improvement in Thalassemia Patients: Literature Review and the Taiwanese Experience

Abstract

The iron chelators deferoxamine (DFO) and deferiprone (L1) have demonstrated their ability to normalize cardiac function in patients with iron overload-induced cardiac disease. However, conventional chelation with subcutaneous DFO fails to prevent iron deposition in two-thirds of thalassemia major patients, placing them at risk of heart failure and its complications. Deferiprone appears to be more effective in cardiac iron removal. The detection and management of heart complications have improved dramatically over the last 7 years. Non invasive techniques of quantifying iron burden via magnetic resonance imaging (MRI) have been validated. A better understanding of cardiac pathophysiology and improved ability to detect at-risk populations are yielding better outcomes and reduced morbidity.

We continue to appraise readily available bedside tools for monitoring thalassemia patients with heart complications, and here we summarize studies from the literature and our own findings. Deferiprone chelation was found to be of statistically significant benefit in upgrading cardiac function and reducing iron accumulation. The use of echocardiography and MRI to closely monitor cardiac functions associated with iron overload complications and mortality has proved quite practical.

Keywords:

• Thalassemia
• Iron chelator
• Cardiac function
• Glutathione S-transferase M1 (GSTM1) gene
• Polymorphism

Notes

*Presented at the 16^th International Conference on Chelation, Limassol, Cyprus, October 25–31, 2006.

47. Peng CT, Fucharoen S, Kontoghiorghes GJ, Tsai CH. Report on the 15th International Conference on Oral Chelation (ICOC) in the Treatment of Thalassemia and Other Diseases in Taichung, Taiwan, 22–26 April, 2005. Hemoglobin 2006; 30(1):63–68.