Van Creveldkliniek, Department of Hematology, University Medical Center Utrecht, Utrecht, The Netherlands.
Sheffield Haemophilia and Thrombosis Centre, Royal Hallamshire Hospital, Sheffield, United Kingdom.
J Hepatol. 2014 Jan;60(1):39-45. doi: 10.1016/j.jhep.2013.08.010. Epub 2013 Aug 23.
BACKGROUND & AIMS: Patients with inherited bleeding disorders are an interesting group to study the long-term course of chronic hepatitis C virus (HCV) infection, because of their uniform mode of infection and reliable follow-up. Our aim was to assess the long-term occurrence of adverse liver-related events in these patients.
The occurrence and determinants of end-stage liver disease (ESLD) were assessed using retrospective data of 863 HCV infected patients with inherited bleeding disorders from the Netherlands and the UK.
Median follow-up since HCV infection was 31 years, while 30% of patients had >35 follow-up years. Nineteen percent of patients spontaneously cleared the virus and 81% developed chronic HCV infection. Of the 700 patients with chronic HCV, 90 (13%) developed ESLD. Hepatocellular carcinoma (HCC) was diagnosed in 3% of patients with chronic HCV, 41% of which occurred in the last six years. Determinants of ESLD development were age at infection (hazard ratio (HR) 1.09 per year increase), HIV co-infection (HR 10.85), history of alcohol abuse (HR 4.34) and successful antiviral treatment (HR 0.14). Of the 487 patients who were still alive at the end of follow-up, 49% did not undergo optimal conventional antiviral treatment.
After over 30 years of HCV infection, ESLD occurred in a significant proportion of patients with inherited bleeding disorders. HCC appears to be an increasing problem. There is a significant potential for both conventional and new antiviral treatment regimens to try and limit ESLD occurrence in the future.
由于遗传性出血性疾病患者的感染方式一致且可进行可靠的随访，因此他们是研究慢性丙型肝炎病毒（HCV）感染长期病程的一个有趣群体。我们的目的是评估这些患者发生不良肝脏相关事件的长期情况。
使用来自荷兰和英国的 863 名遗传性出血性疾病合并 HCV 感染患者的回顾性数据，评估终末期肝病（ESLD）的发生和决定因素。
自 HCV 感染以来的中位随访时间为 31 年，而 30%的患者随访时间超过 35 年。19%的患者自发清除了病毒，81%的患者发展为慢性 HCV 感染。在 700 名患有慢性 HCV 的患者中，90 名（13%）发生了 ESLD。慢性 HCV 患者中有 3%被诊断出患有肝细胞癌（HCC），其中 41%发生在过去六年中。ESLD 发展的决定因素包括感染时的年龄（每年增加的风险比（HR）为 1.09）、HIV 合并感染（HR 10.85）、酒精滥用史（HR 4.34）和成功的抗病毒治疗（HR 0.14）。在随访结束时仍存活的 487 名患者中，49%未接受最佳常规抗病毒治疗。
在 HCV 感染 30 多年后，遗传性出血性疾病患者中出现 ESLD 的比例显著。HCC 似乎是一个日益严重的问题。未来，无论是传统还是新的抗病毒治疗方案，都有很大的潜力尝试限制 ESLD 的发生。
