Borgna-Pignatti C, De Stefano P, Sessa F, Avato F
Med Pediatr Oncol. 1986;14(6):327-8. doi: 10.1002/mpo.2950140610.
The occurrence of hepatocellular carcinoma in a 22-year-old man with thalassemia major is reported. As a result of transfusional hemochromatosis, this patient had already developed diabetes, hypogonadism, heart failure, and the sicca syndrome; he was serum and tissue HBsAg negative. Liver iron concentration measured postmortem was found to be 50 times normal. Multiply transfused patients are at risk of developing hepatocellular carcinoma. Serial measurements of serum alpha-fetoprotein should permit early detection of the tumor and reduce mortality. Preventive measures include early immunisation against hepatitis B virus and prevention of iron accumulation by intensive use of desferrioxamine. Treatment of hemochromatosis-associated hypogonadism with androgens should be considered with caution.
本文报道了一名22岁重型地中海贫血男性患者发生肝细胞癌的病例。由于输血性血色素沉着症,该患者已出现糖尿病、性腺功能减退、心力衰竭和干燥综合征;其血清和组织乙肝表面抗原均为阴性。尸检测得肝脏铁浓度为正常的50倍。多次输血的患者有发生肝细胞癌的风险。定期检测血清甲胎蛋白应有助于早期发现肿瘤并降低死亡率。预防措施包括尽早接种乙肝病毒疫苗以及通过大量使用去铁胺防止铁蓄积。对于血色素沉着症相关的性腺功能减退,使用雄激素治疗时应谨慎考虑。