How early can myocardial iron overload occur in beta thalassemia major?
BACKGROUND
Myocardial siderosis is the most common cause of death in patients with beta thalassemia major(TM). This study aimed at investigating the occurrence, prevalence and severity of cardiac iron overload in a young Chinese population with beta TM.


RESULTS
We analyzed T2* cardiac magnetic resonance (CMR), left ventricular ejection fraction (LVEF) and serum ferritin (SF) in 201 beta TM patients. The median age was 9 years old. Patients received an average of 13 units of blood per year. The median SF level was 4536 ng/ml and 165 patients (82.1%) had SF>2500 ng/ml. Myocardial iron overload was detected in 68 patients (33.8%) and severe myocardial iron overload was detected in 26 patients (12.6%). Twenty-two patients ≤10 years old had myocardial iron overload, three of whom were only 6 years old. No myocardial iron overload was detected under the age of 6 years. Median LVEF was 64% (measured by CMR in 175 patients). Five of 6 patients with a LVEF<56% and 8 of 10 patients with cardiac disease had myocardial iron overload.


CONCLUSIONS
The TM patients under follow-up at this regional centre in China patients are younger than other reported cohorts, more poorly-chelated, and have a high burden of iron overload. Myocardial siderosis occurred in patients younger than previously reported, and was strongly associated with impaired LVEF and cardiac disease. For such poorly-chelated TM patients, our data shows that the first assessment of cardiac T2* should be performed as early as 6 years old.
Department of Hematology, the First Affiliated Hospital of Guangxi Medical University, Nanning, China.
Gaohui
G
Yang
Department of Hematology, the First Affiliated Hospital of Guangxi Medical University, Nanning, China.
Rongrong
R
Liu
Department of Radiology, the First Affiliated Hospital of Guangxi Medical University, Nanning, China.
Peng
P
Peng
Department of Radiology, the First Affiliated Hospital of Guangxi Medical University, Nanning, China.
Liling
L
Long
Department of Hematology, 303rd Hospital of People's Liberation Army, Nanning, China.
Xinhua
X
Zhang
Department of Pediatrics, Women and Children Hospital of Guilin, Guilin, China.
Weijia
W
Yang
Department of Pediatrics, Women and Children Hospital of Yulin, Yulin, China.
Shaohong
S
Tan
Department of Pediatrics, Affiliated Hospital of Youjiang Medical University for Nationality, Baise, China.
Hongfei
H
Pan
Department of Pediatrics, The People's Hospital of Liuzhou, Liuzhou, China.
Xingjiang
X
Long
Cardiovascular Sciences Research Centre, St George's University of London, London, United Kingdom ; Biomedical Research Unit, Royal Brompton Hospital, London, United Kingdom.
Taigang
T
He
Cardiovascular Sciences Research Centre, St George's University of London, London, United Kingdom.
Lisa
L
Anderson
Department of Hematology, the First Affiliated Hospital of Guangxi Medical University, Nanning, China.
Yongrong
Y
Lai
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005293:Ferritins / Q000097:blood; D006331:Heart Diseases / Q000175:diagnosis / Q000378:metabolism / Q000503:physiopathology; D006801:Humans; D007501:Iron / Q000378:metabolism*; D019190:Iron Overload / Q000175:diagnosis / Q000378:metabolism*; D016014:Linear Models; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D015999:Multivariate Analysis; D009206:Myocardium / Q000378:metabolism*; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis; D016277:Ventricular Function, Left; D055815:Young Adult; D017086:beta-Thalassemia / Q000097:blood / Q000175:diagnosis / Q000378:metabolism*
D005293:Ferritins; D007501:Iron