@article {107856, title = {Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study.}, journal = {European journal of haematology}, volume = {95}, year = {2015}, month = {2015 Sep}, pages = {244-53}, abstract = {

OBJECTIVES: The 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.

METHODS: Patients aged >= 10 yr with transfusion-dependent anemias from 11 countries were screened. Data were summarized descriptively, overall and across regions.

RESULTS: Among 925 patients (99.1\% with β-thalassemia major; 98.5\% receiving prior chelation; mean age 19.2 yr), 36.7\% had myocardial iron overload (myocardial T2* <= 20 ms), 12.1\% had low left ventricular ejection fraction. Liver iron concentration (LIC) (mean 25.8 mg Fe/g dw) and serum ferritin (median 3702 ng/mL) were high. Fewer patients in the Middle East (ME; 28.5\%) had myocardial T2* <= 20 ms 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 \< 7 mg 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 \< 7 mg 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.

CONCLUSIONS: Evidence 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.

}, issn = {1600-0609}, doi = {10.1111/ejh.12487}, author = {Yesim Aydinok and John B. Porter and Piga, Antonio and Mohsen Elalfy and Amal El-Beshlawy and Kilin{\c c}, Yurdanur and Vip Viprakasit and Yesilipek, Akif and Dany Habr and Quebe-Fehling, Erhard and Dudley J. Pennell} }