Division of Pediatric Hematology and Oncology, Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung 807378, Taiwan.
School of Post-Baccalaureate Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807378, Taiwan.
Int J Mol Sci. 2023 Aug 10;24(16):12654. doi: 10.3390/ijms241612654.
Thalassemia is a heterogeneous congenital hemoglobinopathy common in the Mediterranean region, Middle East, Indian subcontinent, and Southeast Asia with increasing incidence in Northern Europe and North America due to immigration. Iron overloading is one of the major long-term complications in patients with thalassemia and can lead to organ damage and carcinogenesis. Hepatocellular carcinoma (HCC) is one of the most common malignancies in both transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT). The incidence of HCC in patients with thalassemia has increased over time, as better chelation therapy confers a sufficiently long lifespan for the development of HCC. The mechanisms of iron-overloading-associated HCC development include the increased reactive oxygen species (ROS), inflammation cytokines, dysregulated hepcidin, and ferroportin metabolism. The treatment of HCC in patients with thalassemia was basically similar to those in general population. However, due to the younger age of HCC onset in thalassemia, regular surveillance for HCC development is mandatory in TDT and NTDT. Other supplemental therapies and experiences of novel treatments for HCC in the thalassemia population were also reviewed in this article.
地中海贫血症是一种常见的先天性血红蛋白病，在地中海地区、中东、印度次大陆和东南亚较为普遍，由于移民，北欧和北美地区的发病率也在上升。铁过载是地中海贫血症患者的主要长期并发症之一，可导致器官损伤和癌变。肝细胞癌（HCC）是输血依赖型地中海贫血症（TDT）和非输血依赖型地中海贫血症（NTDT）患者中最常见的恶性肿瘤之一。随着更好的螯合疗法为 HCC 的发展提供了足够长的寿命，地中海贫血症患者的 HCC 发病率也随之增加。铁过载相关 HCC 发展的机制包括增加的活性氧（ROS）、炎症细胞因子、失调的铁调素和亚铁转运蛋白代谢。地中海贫血症患者 HCC 的治疗基本与普通人群相似。然而，由于地中海贫血症患者 HCC 的发病年龄较小，因此 TDT 和 NTDT 患者必须定期监测 HCC 的发展。本文还回顾了地中海贫血症患者 HCC 的其他补充治疗和新型治疗方法的经验。
