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Blood, 1 March 2004, Vol. 103, No. 5, pp. 1934-1936.
Prepublished online as a Blood First Edition Paper on November 20, 2003; DOI 10.1182/blood-2003-06-1919.

Submitted June 16, 2003
Accepted October 9, 2003
Myocardial iron loading in transfusion-dependent thalassemia and sickle-cell disease
John C Wood*, J Michael Tyszka, Susan M Carson, Marvin D Nelson, and Thomas D Coates
Division of Pediatric Cardiology, Childrens Hospital of Los Angeles, University of Southern California School of Medicine, Los Angeles, CA, USA; Department of Biology, California Institute of Technology, Pasadena, CA, USA
Department of Pediatric Hematology, Childrens Hospital of Los Angeles, University of Southern California School of Medicine, Los Angeles, CA, USA
Department of Pediatric Radiology, Childrens Hospital of Los Angeles, University of Southern California School of Medicine, Los Angeles, CA, USA
* Corresponding author; email: jwood{at}chla.usc.edu.
Cardiac T2* is abnormal in approximately 40% of adults with thalassemia major (TM), suggesting myocardial iron deposition, but it is unknown at what age this occurs. To address this question, we measured cardiac T2* and function in 19 young (ages 7-26) TM patients as well as 17 chronically transfused sickle cell anemia (SCA) matched for age, sex, and liver iron content. Cardiac T2* was normal in all of the SCA patients but was low (high iron) in 8/19 TM patients. Abnormal T2* only observed in the TM patients transfused 13 years or more and was correlated with ferritin but not liver iron levels. Cardiac dysfunction was present in 3 of the 8 patients with low T2*. Cardiac T2* changes have a long latency relative to liver iron accumulation. Total transfusional burden is a significant independent risk factor for low cardiac T2* and may partially account for differences observed between SCA and TM patients.

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