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Blood, 1 May 2006, Vol. 107, No. 9, pp. 3436-3441.
Prepublished online as a Blood First Edition Paper on March 7, 2006; DOI 10.1182/blood-2006-02-002394.
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Submitted February 28, 2006
Accepted March 3, 2006
Oral chelators deferasirox and deferiprone for transfusional iron overload in thalassemia major: new data, new questions
Ellis J. Neufeld*
Children's Hospital Boston
* Corresponding author; email: ellis.neufeld{at}childrens.harvard.edu.
For nearly 30 years, patients with transfusional iron overload have depended on nightly deferoxamine infusions for iron chelation. Despite dramatic gains in life expectancy in the deferoxamine era for patients with transfusion-dependent anemias, the leading cause of death for young adults with thalassemia major and related disorders has been cardiac disease from myocardial iron deposition. Strategies to reduce cardiac disease by improving chelation regimens have been of the highest priority. These strategies have included development of novel oral iron chelators to improve compliance, improved assessment of cardiac iron status, and careful epidemiologic assessment of European outcomes with deferiprone, an oral alternative chelator available for about a decade. Each of these strategies is now bearing fruit. The novel oral chelator deferasirox was recently approved by FDA; a randomized clinical trial demonstrates that deferasirox at 20-30 mg/kg/day can maintain or improve hepatic iron in thalassemia as well as deferoxamine. A randomized trial based on cardiac T2* MRI suggests that deferiprone can unload myocardial iron faster than deferoxamine. Retrospective epidemiological data suggest dramatic reductions in cardiac events and mortality in Italian subjects exposed to deferiprone, compared to deferoxamine. These developments herald a new era for iron chelation, but many unanswered questions remain.
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