Ouederni Monia, Ben Khaled Monia, Mellouli Fethi, Ben Fraj Elhem, Dhouib Nawel, Yakoub Ismehen Ben, Abbes Selem, Mnif Nejla, Bejaoui Mohamed
Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia.
Pediatric Immuno-Hematology Unit, Bone Marrow Transplantation Center Tunis, Tunis, Tunisia.
Ann Hematol. 2017 Jan;96(1):133-139. doi: 10.1007/s00277-016-2841-5. Epub 2016 Oct 12.
Thalassemia is a common genetic disorder in Tunisia. Early iron concentration assessment is a crucial and challenging issue. Most of annual deaths due to iron overload occurred in underdeveloped regions of the world. Limited access to liver and heart MRI monitoring might partially explain these poor prognostic results. Standard software programs are not available in Tunisia. This study is the first to evaluate iron overload in heart and liver using the MRI T2* with excel spreadsheet for post processing. Association of this MRI tool results to serum ferritin level, and echocardiography was also investigated. One hundred Tunisian-transfused thalassemia patients older than 10 years (16.1 ± 5.2) were enrolled in the study. The mean myocardial iron concentration (MIC) was 1.26 ± 1.65 mg/g dw (0.06-8.32). Cardiac T2* (CT2*) was under 20 ms in 30 % of patients and under 10 ms in 21 % of patients. Left ventricular ejection function was significantly lower in patients with CT2* <10 ms. Abnormal liver iron concentration (LIC >3 mg/g dw) was found in 95 % of patients. LIC was over 15 mg/g dw in 25 % of patients. MIC was more correlated than CT2* to LIC and serum ferritin. Among patients with SF <1000 μg/l, 13 % had CT2* <20 ms. Our data showed that 30 % of the Tunisian thalassemia major patients enrolled in this cohort had myocardial iron overload despite being treated by iron chelators. SF could not reliably predict iron overload in all thalassemia patients. MRI T2* using excel spreadsheet for routine follow-up of iron overload might improve the prognosis of thalassemia major patients in developing countries, such as Tunisia, where standard MRI tools are not available or expensive.
地中海贫血在突尼斯是一种常见的遗传性疾病。早期铁浓度评估是一个关键且具有挑战性的问题。世界上大多数因铁过载导致的年度死亡发生在欠发达地区。肝脏和心脏MRI监测的可及性有限可能部分解释了这些预后不良的结果。突尼斯没有标准的软件程序。本研究首次使用MRI T2结合Excel电子表格进行后处理来评估心脏和肝脏的铁过载情况。还研究了该MRI工具结果与血清铁蛋白水平及超声心动图的相关性。本研究纳入了100例年龄超过10岁(16.1±5.2岁)的突尼斯输血性地中海贫血患者。心肌铁浓度(MIC)平均为1.26±1.65mg/g干重(0.06 - 8.32)。30%的患者心脏T2(CT2*)低于20ms,21%的患者低于10ms。CT2*<10ms的患者左心室射血功能显著降低。95%的患者肝脏铁浓度异常(LIC>3mg/g干重)。25%的患者LIC超过15mg/g干重。MIC与LIC和血清铁蛋白的相关性比CT2更强。在血清铁蛋白(SF)<1000μg/l的患者中,13%的患者CT2<20ms。我们的数据显示,尽管接受了铁螯合剂治疗,但该队列中30%的突尼斯重型地中海贫血患者仍有心肌铁过载。SF不能可靠地预测所有地中海贫血患者的铁过载情况。在突尼斯等发展中国家,标准MRI工具不可用或昂贵,使用Excel电子表格进行铁过载常规随访的MRI T2*可能会改善重型地中海贫血患者的预后。