PUMA
Istituto di Fisiologia Clinica     
Pepe A., Lombardi M., Positano V., Cracolici E., Capra M., Malizia R., Prossomariti L., De Marchi D., Midiri M., Maggio A. Evaluation of the efficacy of oral deferiprone in beta-thalassemia major by multislice multiecho T2*. In: European Journal of Haematology, vol. 76 (3) pp. 183 - 192. Blackwell, 2006.
 
 
Abstract
(English)
Objectives: Oral deferiprone (L1) appears to be promising in the treatment of beta-thalassemia major (TM) patients. T2* magnetic resonance imaging (MRI) with a single measurement in the mid-ventricular septum was validated as a quantitative evaluation of myocardial iron overload. Previous studies suggested a marked heterogeneity of iron distribution in the myocardium. We set up a multislice multiecho T2* MRI for the detection of this heterogeneity. The aim of our study was to investigate differences between the L1 vs. the subcutaneous desferrioxamine (DF)-treated patients using this new approach. Methods: Thirty-six beta-TM patients (age 29 8 yr) underwent MRI. Eighteen patients received long-term L1, and 18 other patients matched for age and sex received DF. T2* multiecho sequences on three short axis views of the left ventricle were obtained and analyzed by custom-made software. In each slice, the myocardium was automatically segmented into four segments. Cine-dynamic images were also obtained to evaluate biventricular function. Results: For multislice T2* technique, the coefficient of variation for intra- and inter-observer, and inter-study reproducibility was 3.9%, 4.7%, and 5.5%, respectively. The global heart T2* value was significantly higher in the L1 vs. DF group (35 7 vs. 27 2 ms; P = 0.02). The number of segments with normal T2* value (>20 ms) was significantly higher in the L1 vs. the DF group (11 1 vs. 8 5 segments; P = 0.03). We did not detect significant differences in biventricular function parameters. Conclusions: This new approach confirms that L1 could be more effective than DF in removal of myocardial iron.
URL: http://www.blackwell-synergy.com/doi/abs/10.1111/j.1600-0609.2005.00587.x
Subject beta-thalassemia;
Multislice multiecho T2
Chelation treatment
Magnetic resonance imaging
Iron overload


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