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

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.

• Authors: Yesim Aydinok, John B Porter, Antonio Piga, Mohsen Elalfy, Amal El-Beshlawy, Yurdanur Kilinç, Vip Viprakasit, Akif Yesilipek, Dany Habr, Erhard Quebe-Fehling, Dudley J Pennell
• Journal: European journal of haematology (Eur J Haematol) Vol. 95 Issue 3 Pg. 244-53 (Sep 2015) ISSN: 1600-0609 [Electronic] England
• PMID: 25418187 (Publication Type: Clinical Trial, Phase II, Journal Article, Multicenter Study, Randomized Controlled Trial, Research Support, Non-U.S. Gov't)
• Copyright: © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Chemical References

• Iron Chelating Agents
• Iron
• Deferoxamine

Topics

• Adolescent
• Adult
• Aged
• Aged, 80 and over
• Anemia (complications, therapy)
• Child
• Deferoxamine (therapeutic use)
• Female
• Humans
• Iron (metabolism)
• Iron Chelating Agents (therapeutic use)
• Iron Overload (drug therapy, epidemiology, etiology, metabolism)
• Liver (metabolism, pathology)
• Male
• Middle Aged
• Myocardium (metabolism, pathology)
• Prevalence
• Tissue Distribution
• Transfusion Reaction
• Young Adult