Evaluation of the efficacy of oral deferiprone in beta-thalassemia major by multislice multiecho T2*.
OBJECTIVE
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.
Pepe
Alessia
A
MRI Laboratory, Institute of Clinical Physiology, CNR, Pisa, Italy. alepepe@email.it
Lombardi
Massimo
M
Positano
Vincenzo
V
Cracolici
Eliana
E
Capra
Marcello
M
Malizia
Roberto
R
Prossomariti
Luciano
L
De Marchi
Daniele
D
Midiri
Massimo
M
Maggio
Aurelio
A
D000328:Adult; D000077543:Deferiprone; D003676:Deferoxamine / Q000008:administration & dosage; D005260:Female; D006801:Humans; D019190:Iron Overload / Q000175:diagnosis / Q000188:drug therapy; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008280:Magnetics; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism; D011728:Pyridones / Q000008:administration & dosage*; D017086:beta-Thalassemia / Q000150:complications / Q000188:drug therapy* / Q000473:pathology
D011728:Pyridones; D000077543:Deferiprone; D003676:Deferoxamine