Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA.
Department of Medicine and Center for Research on Healthcare, University of Pittsburgh Medical Center, Pittsburgh, PA.
Blood Adv. 2024 Nov 26;8(22):5767-5772. doi: 10.1182/bloodadvances.2024014350.
Hemophilia is an X-linked congenital bleeding disorder for which factor replacement is life-saving but complicated by the sequelae of chronic hepatitis C virus (HCV) infection acquired decades ago. Although antiviral therapy clears HCV and reduces end-stage liver disease (ESLD), it may not reverse cirrhosis or prevent hepatocellular cancer (HCC). This was a retrospective cohort study of 121 men with hemophilia and HCV infection cared for at the Hemophilia Center of Western Pennsylvania to determine the incidence and predictors of ESLD and HCC. ESLD and HCC predictors were analyzed using Fisher exact test, and HCV-associated outcomes by Kaplan-Meier time-to-event and Cox proportional hazards regression analyses. At a mean 54 years (36-80) duration of HCV, ESLD occurred in 24 (19.8%), 0.365 per 100 person-years (py); and HCC in 7 (5.8%), 0.106 per 100 py. All 46 (38.0%) alive when HCV antiviral therapy became available, received it. Overall, 31 (25.6%) were HIV+. The leading causes of death were ESLD in 11 (32.3%), bleeding in 9 (26.5%), and HCC in 6 (17.6%). Major risk factors for ESLD included platelets <100 × 103/μL (odds ratio [OR], 6.009; P = .012) and HIV infection (OR, 3.883; P = .001). The major predictors of HCC were ESLD (OR, 11.476; P = .003) and platelets <100 000/μL (OR, 6.159; P = .014). No antiviral-treated patient developed ESLD, P = .001. For men with hemophilia, the sequelae of chronic HCV infection were significant. The major risk factors for ESLD were platelets <100 000/μL and HIV infection. Despite antiviral therapy, ESLD is the most significant predictor of HCC, and ESLD is the leading cause of death.
血友病是一种 X 连锁的先天性出血性疾病，其救命疗法是补充因子，但由于几十年前感染了慢性丙型肝炎病毒 (HCV)，导致其出现了一系列后遗症。虽然抗病毒治疗可以清除 HCV 并减少终末期肝病 (ESLD)，但它可能无法逆转肝硬化或预防肝细胞癌 (HCC)。这是一项回顾性队列研究，共纳入了 121 名在宾夕法尼亚州西部血友病中心接受治疗的患有 HCV 感染的血友病男性患者，旨在确定 ESLD 和 HCC 的发生率和预测因素。采用 Fisher 确切检验分析 ESLD 和 HCC 的预测因素，采用 Kaplan-Meier 时间事件和 Cox 比例风险回归分析分析 HCV 相关结局。在 HCV 持续时间的平均值为 54 年（36-80 岁）的情况下，24 例（19.8%）发生 ESLD，每 100 人年发生 0.365 例；7 例（5.8%）发生 HCC，每 100 人年发生 0.106 例。所有 46 名（38.0%）在 HCV 抗病毒治疗可用时仍存活，他们均接受了治疗。总体而言，31 名（25.6%）患者 HIV 阳性。死亡的主要原因是 ESLD（11 例，32.3%）、出血（9 例，26.5%）和 HCC（6 例，17.6%）。ESLD 的主要危险因素包括血小板<100×103/μL（比值比 [OR]，6.009；P=0.012）和 HIV 感染（OR，3.883；P=0.001）。HCC 的主要预测因素是 ESLD（OR，11.476；P=0.003）和血小板<100000/μL（OR，6.159；P=0.014）。没有接受抗病毒治疗的患者发生 ESLD，P=0.001。对于血友病男性患者而言，慢性 HCV 感染的后遗症是显著的。ESLD 的主要危险因素是血小板<100000/μL 和 HIV 感染。尽管进行了抗病毒治疗，但 ESLD 仍是 HCC 的最重要预测因素，ESLD 也是死亡的主要原因。
