Rezaei-Kalantari Kiara, Meftah Elahe, Tofighi Saeed, Khalaj Kamand, Zoroufian Arezou, Motevalli Marzieh, Inusah Bihinaa Mohammed, Omidi Negar, Ghorashi Seyyed Mojtaba
Rajaie Cardiovascular Medical and Research Center Iran University of Medical Sciences, Tehran, Iran.
Students' Scientific Research Center Tehran University of Medical Sciences, Tehran, Iran.
Cardiol Res Pract. 2024 Jul 24;2024:8842016. doi: 10.1155/2024/8842016. eCollection 2024.
Patients with -thalassemia major depend on lifelong transfusion, resulting in tissue iron overload. This longitudinal retrospective observational study aims to assess myocardial and liver iron overload using magnetic resonance imaging (MRI) and investigate the lag between myocardial and liver iron unloading in -thalassemia patients undergoing chelation therapy.
Beta-thalassemia major patients with at least two MRI studies between 2016 and 2020 were enrolled. Myocardial and liver iron overload were defined as T2 less than 20 and 2.1, respectively. Outcomes included mortality, myocardial and liver T2 changes, and systolic dysfunction assessed by cardiac MRI.
Fifty-five patients with a mean age of 24.62 ± 7.94 years, a mean follow-up duration of 24.3 ± 12.9 months, and a mean ferritin level of 1475.75 ± 771.12 ng/mL were enrolled. All of the abovementioned patients only took deferoxamine as the iron-chelating medication. Mortality occurred in three patients (5.5%) during follow-up. Liver T2 significantly increased ( value <0.05), while myocardial T2 showed a nonsignificant increase. Iron unloading of the myocardium was not significantly different from that of the liver and did not result in a significant lag (56% vs. 44%; value = 0.419). Baseline myocardial T2 correlated with extramedullary hematopoiesis, weekly number of deferoxamine injections ( value <0.01), timing between the transfusions, and serum ferritin ( value <0.05).
Liver T2 reduced during deferoxamine chelation therapy, while myocardial T2 remained unchanged. No significant lag was observed between myocardial and liver iron unloading. Further studies are required to elucidate these findings.
重型β地中海贫血患者依赖终身输血，导致组织铁过载。这项纵向回顾性观察研究旨在使用磁共振成像（MRI）评估心肌和肝脏铁过载，并调查接受螯合治疗的β地中海贫血患者心肌和肝脏铁清除之间的滞后情况。
纳入2016年至2020年间至少进行过两次MRI检查的重型β地中海贫血患者。心肌和肝脏铁过载分别定义为T2小于20和2.1。观察指标包括死亡率、心肌和肝脏T2变化以及通过心脏MRI评估的收缩功能障碍。
共纳入55例患者，平均年龄24.62±7.94岁，平均随访时间24.3±12.9个月，平均铁蛋白水平1475.75±771.12 ng/mL。上述所有患者仅使用去铁胺作为铁螯合剂。随访期间有3例患者（5.5%）死亡。肝脏T2显著增加（P值<0.05），而心肌T2虽有增加但无统计学意义。心肌的铁清除与肝脏无显著差异，也未导致明显滞后（56%对44%；P值=0.419）。基线心肌T2*与髓外造血、每周去铁胺注射次数（P值<0.01）、输血间隔时间以及血清铁蛋白（P值<0.05）相关。
去铁胺螯合治疗期间肝脏T2降低，而心肌T2保持不变。心肌和肝脏铁清除之间未观察到明显滞后。需要进一步研究以阐明这些发现。
