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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.


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RED CELLS
Brief report

Myocardial iron loading in transfusion-dependent thalassemia and sickle cell disease

John C. Wood, J. Michael Tyszka, Susan Carson, Marvin D. Nelson, and Thomas D. Coates

From the Divisions of Pediatric Cardiology and Hematology and the Department of Pediatric Radiology, Childrens Hospital Los Angeles, Los Angeles, CA; and Department of Biology, Biological Imaging Center, California Institute of Technology, Pasadena, CA.

Cardiac T2* (magnetic resonance imaging relaxation parameter) is abnormally low 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 patients (aged 7-26 years) with TM as well as 17 patients receiving long-term transfusions for 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 of 19 TM patients. Abnormal T2* was observed only in the TM patients receiving transfusions for 13 years or longer 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 patients with SCA and TM.


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