Fianza Pandji Irani, Rahmawati Anita, Widihastha Sri Hudaya, Afifah Shofura, Ghozali Mohammad, Indrajaya Andre, Pratama Dilli Marayuzan Akbar, Prasetya Dimmy, Sihite Teddy Arnold, Syamsunarno Mas Rizky A A, Setiabudi Djatnika, Fucharoen Suthat, Panigoro Ramdan
Department of Internal Medicine, Division of Hematology and Medical Oncology, Faculty of Medicine, Universitas Padjadjaran/Hasan Sadikin General Hospital, Bandung, West Java, Indonesia.
Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, Bandung, West Java, Indonesia.
Anemia. 2021 Apr 15;2021:5581831. doi: 10.1155/2021/5581831. eCollection 2021.
Thalassemia is a genetic disease caused by disruption of globin chain synthesis leading to severe anemia and thus regular blood transfusion is necessary. However, there have been known transfusions-related consequences, including iron overload and multi-organ damage. The aims of this study were to evaluate liver and cardiac function in youth and adult transfusion-dependent Indonesian thalassemic patients and to assess its correlation with serum ferritin levels, as well as T2 magnetic resonance imaging (MRI). Transfusion-dependent thalassemic (TDT) outpatients ( = 66; mean age, 21.5 ± 7.2 years) were carried out for the complete assessment consisting of blood test including liver enzyme and serum ferritin, followed by electrocardiography (ECG) and echocardiography. Subjects were also divided by serum ferritin levels into three groups: < 2500 ng/mL, 2500-5000 ng/mL, and >5000 ng/mL. Additionally, subgroup analysis in patients with T2 MRI assessment was conducted. In terms of age of first blood transfusion, subjects with ferritin >5000 ng/mL were the youngest among others. The alanine aminotransferase (ALT) levels in group with serum ferritin >5000 ng/mL were significantly higher than those of the group with serum ferritin <2500 ng/mL. Additionally, youth and adult TDT patients whose serum ferritin >5000 ng/mL had significantly lower tricuspid annular plane systolic excursion (TAPSE) when compared with those who had serum ferritin <2500 ng/mL. Similarly, TAPSE in patients with moderate cardiac siderosis based on cardiac T2 MRI was significantly lower than those without cardiac siderosis. There was significant, but only moderate correlation between serum ferritin and cardiac T2 MRI. Based on these findings, it is important to routinely monitor iron accumulation-related complications, including liver and cardiac damage in youth and adult TDT patients.
地中海贫血是一种由珠蛋白链合成中断引起的遗传性疾病，会导致严重贫血，因此需要定期输血。然而，输血会带来一些已知的后果，包括铁过载和多器官损伤。本研究的目的是评估印度尼西亚依赖输血的青少年和成年地中海贫血患者的肝脏和心脏功能，并评估其与血清铁蛋白水平以及T2磁共振成像（MRI）的相关性。对依赖输血的地中海贫血（TDT）门诊患者（n = 66；平均年龄，21.5±7.2岁）进行了全面评估，包括血液检查，其中包括肝酶和血清铁蛋白检测，随后进行心电图（ECG）和超声心动图检查。受试者还根据血清铁蛋白水平分为三组：<2500 ng/mL、2500 - 5000 ng/mL和>5000 ng/mL。此外，对进行了T2 MRI评估的患者进行了亚组分析。在首次输血年龄方面，铁蛋白>5000 ng/mL的受试者是最年轻的。血清铁蛋白>5000 ng/mL组的丙氨酸氨基转移酶（ALT）水平显著高于血清铁蛋白<2500 ng/mL组。此外，血清铁蛋白>5000 ng/mL的青少年和成年TDT患者与血清铁蛋白<2500 ng/mL的患者相比，三尖瓣环平面收缩期位移（TAPSE）显著更低。同样，基于心脏T2 MRI诊断为中度心脏铁沉着症的患者的TAPSE显著低于无心脏铁沉着症的患者。血清铁蛋白与心脏T2 MRI之间存在显著但仅为中度的相关性。基于这些发现，常规监测青少年和成年TDT患者与铁蓄积相关的并发症，包括肝脏和心脏损伤非常重要。
