U.O.C. Ematologia-Emoglobinopatie, Arnas Civico, Palermo, Italy.
Clin Drug Investig. 2010;30(4):267-73. doi: 10.2165/11534480-000000000-00000.
We report the long-term effects of deferasirox 10-30 mg/kg/day on cardiac iron overload in a case series of five patients with transfusion-dependent beta-thalassaemia major who underwent up to 5 years of chelation therapy. Iron overload was monitored by multislice multi-echo T2* magnetic resonance imaging (MRI). Overall, T2* MRI showed a decrease from baseline in cardiac iron levels in all patients during treatment with deferasirox (baseline T2* levels 13-24 ms; final T2* levels 21-41 ms). The improvement in T2* values correlated with maintenance of left ventricular ejection fraction (LVEF) [baseline LVEF values 56-61%; final LVEF values 57-70%]. Deferasirox chelation treatment regimen was well tolerated and adherence to the regimen was good. In conclusion, this case series suggests that deferasirox may decrease cardiac iron overload and maintain stable LVEF over the long term. Moreover, it emphasizes the importance of T2* multislice multi-echo MRI in the monitoring of cardiac iron overload.
我们报告了长期使用地拉罗司 10-30mg/kg/天对 5 例输血依赖型重型β地中海贫血患者心脏铁过载的影响，这些患者接受了长达 5 年的螯合治疗。心脏铁过载通过多层多回波 T2磁共振成像（MRI）进行监测。总的来说，地拉罗司治疗期间，所有患者的心脏铁水平均较基线下降（基线 T2水平为 13-24ms；最终 T2水平为 21-41ms）。T2值的改善与左心室射血分数（LVEF）的维持相关[基线 LVEF 值为 56-61%；最终 LVEF 值为 57-70%]。地拉罗司螯合治疗方案耐受良好，患者的依从性也较好。总之，本病例系列研究表明，地拉罗司可能会减少心脏铁过载，并在长期内维持稳定的 LVEF。此外，它强调了 T2*多层多回波 MRI 在心脏铁过载监测中的重要性。
