Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study

ObjectivesThe randomized comparison of deferasirox to deferoxamine for myocardial iron removal in patients with transfusion-dependent anemias (CORDELIA) gave the opportunity to assess relative prevalence and body distribution of iron overload in screened patients. MethodsPatients aged 10yr with transfusion-dependent anemias from 11 countries were screened. Data were summarized descriptively, overall and across regions. ResultsAmong 925patients (99.1% with -thalassemia major; 98.5% receiving prior chelation; mean age 19.2yr), 36.7% had myocardial iron overload (myocardial T2* 20ms), 12.1% had low left ventricular ejection fraction. Liver iron concentration (LIC) (mean 25.8mg Fe/g dw) and serum ferritin (median 3702ng/mL) were high. Fewer patients in the Middle East (ME; 28.5%) had myocardial T2* 20ms vs. patients in the West (45.9%) and Far East (FE, 40.9%). Patients in the West had highest myocardial iron burden, but lowest LIC (26.9% with LIC <7mg Fe/g dw) and serum ferritin. Among patients with normal myocardial iron, a higher proportion of patients from the ME and FE had LIC 15 than <7mg Fe/g dw (ME, 56.7% vs. 17.2%; FE, 78.6% vs. 7.8%, respectively), a trend which was less evident in the West (44.6% vs. 33.9%, respectively). Transfusion and chelation practices differed between regions. ConclusionsEvidence of substantial myocardial and liver iron burden across regions revealed a need for optimization of effective, convenient iron chelation regimens. Significant regional variation exists in myocardial and liver iron loading that are not well explained; improved understanding of factors contributing to differences in body iron distribution may be of clinical benefit.