Status:
Ready to upload
Record number:
1188
Adverse Occurrence type:
MPHO Type:
Estimated frequency:
Age >10 years, >50 red cell transfusions and >10 units per year
Time to detection:
>50 units red blood cell transfusions
Alerting signals, symptoms, evidence of occurrence:
Among 925 patients (99.1% with β thalassemia major; 98.5% receiving prior chelation; mean age 19.2 years), 36.7% had myocardial iron overload (myocardial T2* ≤20ms), 12.1% had low left ventricular ejection fraction. 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* ≤20ms versus 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).
Demonstration of imputability or root cause:
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.
Imputability grade:
3 Definite/Certain/Proven
Groups audience:
Keywords:
Suggest new keywords:
splenectomy, siderosis, iron overload, myocardial iron overload, chelation,
Thalassemia, anemia
Suggest references:
Yesim Aydinok, John B Porter, Antonio Piga, Mohsen Elalfy, Amal El-Beshlawy, Yurdanur Kilinç, Vip Viprakasit, Akif Yesilipek, Dany Habr, Erhard Quebe-Fehling and Dudley J Pennell (2014). Prevalence and Distribution of Iron Overload in Patients with Transfusion-dependent Anemias Differs across Geographic Regions: Results from the CORDELIA Study. Euorpean J Haematology Accepted manuscript online: 24 NOV 2014, DOI: 10.1111/ejh.12487.
Expert comments for publication:
No meaningful correlation between myocardial siderosis and liver iron concentration. Majority of Far East patients not splenectomized---splenectomy associated with increased myocardial siderosis.