Holmström M, Nangarhari A, Öhman J, Duberg A-S, Majeed A, Aleman S
Coagulation Unit, Department of Medicine Solna, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden.
Department of Infectious Diseases, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden.
Haemophilia. 2016 Nov;22(6):e494-e501. doi: 10.1111/hae.13020. Epub 2016 Oct 5.
Hepatitis C virus (HCV) infection is common in patients with inherited bleeding disorders treated with clotting factor concentrates prior to the introduction of viral inactivation of these products. The long-term consequences of hepatitis C infection in Swedish patients are not fully understood.
To examine the impact of HCV infection on liver-related morbidity and mortality in Swedish patients with inherited bleeding disorders.
We retrospectively collected data on 183 patients with inherited bleeding disorders infected with HCV who attended the Coagulation Unit at Karolinska University Hospital, Sweden. Data regarding end-stage liver disease (ESLD), defined as presence of ascites, encephalopathy, variceal bleeding, hepatocellular carcinoma or liver-related death, were collected from the patient records and the national registers.
The median follow-up time was 35.9 years (IQR 29.0-41.2). A total of 41% had achieved sustained virological response (SVR) after treatment. In total, 14.2% developed ESLD at the median age of 52.6 years (IQR 46.5-64.7). Nineteen (35.8%) of all deaths were due to liver-related causes. Co-infection with human immunodeficiency virus (HIV), older age at time of infection and severe form of bleeding disorder was associated with higher risk of developing ESLD, while SVR was a strong protective factor.
This study demonstrated that liver-related morbidity and mortality was significant in patients with bleeding disorders and HCV infection in Sweden. Patients with HCV-infection should be candidates for treatment with the new highly effective antiviral drugs, since SVR proved to be a strong protective factor.
在凝血因子浓缩物进行病毒灭活之前，丙型肝炎病毒（HCV）感染在接受该治疗的遗传性出血性疾病患者中很常见。瑞典患者丙型肝炎感染的长期后果尚未完全明了。
研究HCV感染对瑞典遗传性出血性疾病患者肝脏相关发病率和死亡率的影响。
我们回顾性收集了183例感染HCV的遗传性出血性疾病患者的数据，这些患者在瑞典卡罗林斯卡大学医院凝血科就诊。从患者病历和国家登记处收集了关于终末期肝病（ESLD）的数据，ESLD定义为存在腹水、肝性脑病、静脉曲张出血、肝细胞癌或肝脏相关死亡。
中位随访时间为35.9年（四分位间距29.0 - 41.2）。共有41%的患者在治疗后实现了持续病毒学应答（SVR）。总计14.2%的患者在中位年龄52.6岁（四分位间距46.5 - 64.7）时发展为ESLD。所有死亡病例中有19例（35.8%）是由肝脏相关原因导致的。合并人类免疫缺陷病毒（HIV）感染、感染时年龄较大以及严重形式的出血性疾病与发展为ESLD的较高风险相关，而SVR是一个强有力的保护因素。
本研究表明，在瑞典，出血性疾病合并HCV感染的患者肝脏相关发病率和死亡率较高。由于SVR被证明是一个强有力的保护因素，HCV感染患者应成为使用新型高效抗病毒药物治疗的对象。
