Wahidiyat Pustika Amalia, Liauw Felix, Sekarsari Damayanti, Putriasih Siti Ayu, Berdoukas Vasili, Pennell Dudley J
a Pediatric Hematology-Oncology Division, Department of Child Health, Faculty of Medicine , Universitas Indonesia - Dr Cipto Mangunkusumo General Hospital , Jakarta , Indonesia.
b Department of Child Health, Faculty of Medicine , Universitas Indonesia - Dr Cipto Mangunkusumo General Hospital , Jakarta , Indonesia.
Hematology. 2017 Sep;22(8):501-507. doi: 10.1080/10245332.2017.1292614. Epub 2017 Feb 20.
Recent advancements have promoted the use of T2* magnetic resonance imaging (MRI) in the non-invasive detection of iron overload in various organs for thalassemia major patients. This study aims to determine the iron load in the heart and liver of patients with thalassemia major using T2* MRI and to evaluate its correlation with serum ferritin level and iron chelation therapy.
This cross-sectional study included 162 subjects diagnosed with thalassemia major, who were classified into acceptable, mild, moderate, or severe cardiac and hepatic iron overload following their T2* MRI results, respectively, and these were correlated to their serum ferritin levels and iron chelation therapy.
The study found that 85.2% of the subjects had normal cardiac iron stores. In contrast, 70.4% of the subjects had severe liver iron overload. A significant but weak correlation (r = -0.28) was found between cardiac T2* MRI and serum ferritin, and a slightly more significant correlation (r = 0.37) was found between liver iron concentration (LIC) and serum ferritin.
The findings of this study are consistent with several other studies, which show that patients generally manifest with liver iron overload prior to cardiac iron overload. Moreover, iron accumulation demonstrated by T2* MRI results also show a significant correlation to serum ferritin levels.
This is the first study of its kind conducted in Indonesia, which supports the fact that T2* MRI is undoubtedly valuable in the early detection of cardiac and hepatic iron overload in thalassemia major patients.
近期的进展推动了T2磁共振成像(MRI)在重型地中海贫血患者各器官铁过载无创检测中的应用。本研究旨在利用T2 MRI测定重型地中海贫血患者心脏和肝脏的铁负荷,并评估其与血清铁蛋白水平及铁螯合治疗的相关性。
这项横断面研究纳入了162例确诊为重型地中海贫血的受试者,根据其T2* MRI结果分别分为心脏和肝脏铁过载可接受、轻度、中度或重度组,并将这些结果与其血清铁蛋白水平和铁螯合治疗进行相关性分析。
研究发现,85.2%的受试者心脏铁储存正常。相比之下,70.4%的受试者存在严重的肝脏铁过载。心脏T2* MRI与血清铁蛋白之间存在显著但较弱的相关性(r = -0.28),肝脏铁浓度(LIC)与血清铁蛋白之间的相关性稍强(r = 0.37)。
本研究结果与其他多项研究一致,这些研究表明,患者通常在出现心脏铁过载之前就表现出肝脏铁过载。此外,T2* MRI结果显示的铁蓄积也与血清铁蛋白水平显著相关。
这是在印度尼西亚进行的同类研究中的首例,支持了T2* MRI在重型地中海贫血患者心脏和肝脏铁过载早期检测中无疑具有重要价值这一事实。