Karimi Mehran, Amirmoezi Fatemeh, Haghpanah Sezaneh, Ostad Seyedpouria, Lotfi Mehrzad, Sefidbakht Sepideh, Rezaian Shahed
a Hematology Research Center, Shiraz University of Medical Sciences , Shiraz , Iran.
b Radiology Research Center, Shiraz University of Medical Sciences , Shiraz , Iran.
Pediatr Hematol Oncol. 2017 Aug;34(5):292-297. doi: 10.1080/08880018.2017.1400135. Epub 2017 Nov 30.
Beta-thalassemia intermediate is a genetic disease that is milder than beta-thalassemia major. The T2 magnetic resonance imaging (MRI) technique is currently the gold standard for iron load detection. However, it is expensive and needs an expert radiologist to report findings. Therefore, we conducted this study to determine an optimal cut-off value of ferritin in proportion to T2 MRI of liver and measurement of liver iron concentration for early detection of hepatic iron overload in Beta-thalassemia intermediate patients.
This cross-sectional study was conducted on 108 patients with Beta-thalassemia intermediate who referred to tertiary hospital, Shiraz, Iran. Serum ferritin, hepatic T2 MRI, and liver iron concentration were assessed. Receiver operator characteristic was used to determine the sensitivity and specificity of cut-off value.
Serum ferritin levels showed a statistically significant negative correlation with T2 hepatic MRI (r = -0.290, p value =.003) and positive correlation with liver iron concentration (r = 0.426, p value <.001) in the patients with Beta-thalassemia intermediate. According to the receiver operator characteristic, the best cut-off value for ferritin to show early diagnosis of liver iron overload was 412 ng/mL. Calculated sensitivities and specificities were 0.78 and 0.82 for T2 MRI and 0.76 and 0.86 for liver iron concentration, respectively.
Serum ferritin levels of around 450 ng/mL might be considered as a cut-off point to evaluate hepatic iron overload before using expensive, not readily available T2 MRI. This level of serum ferritin could be considered for starting iron chelation therapy in patients with Beta-thalassemia intermediate in areas where T2 MRI is not available.
中间型β地中海贫血是一种比重型β地中海贫血症状较轻的遗传性疾病。T2磁共振成像(MRI)技术是目前检测铁负荷的金标准。然而,该技术成本高昂,且需要专业放射科医生报告检查结果。因此,我们开展了本研究,以确定与肝脏T2 MRI及肝脏铁浓度测量值相对应的铁蛋白最佳临界值,用于早期检测中间型β地中海贫血患者的肝脏铁过载情况。
本横断面研究纳入了108例转诊至伊朗设拉子三级医院的中间型β地中海贫血患者。对患者进行血清铁蛋白、肝脏T2 MRI及肝脏铁浓度评估。采用受试者工作特征曲线确定临界值的敏感性和特异性。
在中间型β地中海贫血患者中,血清铁蛋白水平与肝脏T2 MRI呈显著负相关(r = -0.290,p值 = 0.003),与肝脏铁浓度呈正相关(r = 0.426,p值 < 0.001)。根据受试者工作特征曲线,铁蛋白用于早期诊断肝脏铁过载的最佳临界值为412 ng/mL。T2 MRI的计算敏感性和特异性分别为0.78和0.82,肝脏铁浓度的计算敏感性和特异性分别为0.76和0.86。
在使用昂贵且不易获得的T2 MRI之前,血清铁蛋白水平约450 ng/mL可作为评估肝脏铁过载的临界值。在无法进行T2 MRI检查的地区,对于中间型β地中海贫血患者,可考虑以此血清铁蛋白水平作为开始铁螯合治疗的依据。