Yesim Aydinok1, John B Porter2, Antonio Piga3, Mohsen Elalfy4, Amal El-Beshlawy5, Yurdanur Kilinç6, Vip Viprakasit7, Akif Yesilipek8, Dany Habr9, Erhard Quebe-Fehling10, Dudley J Pennell11. 1. Ege University Hospital, Izmir, Turkey. 2. University College London, London, UK. 3. University of Turin, Turin, Italy. 4. Ain Shams University, Cairo, Egypt. 5. Cairo University, Cairo, Egypt. 6. Cukurova University Medical Faculty, Adana, Turkey. 7. Siriraj Hospital, Mahidol University, Bangkok, Thailand. 8. Akdeniz University, Antalya, Turkey. 9. Novartis Pharmaceuticals, East Hanover, NJ, USA. 10. Novartis Pharma AG, Basel, Switzerland. 11. NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital, London, UK.
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.
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: Tianle Cao; Nan Wang; Alan C Kwan; Hsu-Lei Lee; Xianglun Mao; Yibin Xie; Kim-Lien Nguyen; Caroline M Colbert; Fei Han; Pei Han; Hui Han; Anthony G Christodoulou; Debiao Li Journal: Magn Reson Med Date: 2022-06-17 Impact factor: 3.737