Abstract

Portal vein thrombosis (PVT) is a rare but severe condition. Several risk factors predispose to PVT. However, it remains unclear to which degree thrombophilia contributes to the risk of PVT and whether PVT patients should be routinely referred for thrombophilia testing. The aim of the present study was to investigate the prevalence of thrombophilia in PVT patients to clarify the relevance of thrombophilia testing in PVT patients. Clinical data and results from thrombophilia investigations were systematically obtained from all PVT patients referred to Centre for Hemophilia and Thrombosis, Aarhus University Hospital, Denmark for thrombophilia testing between 1^st of January 2010 and 31^st of December 2018 (n = 93). The investigated thrombophilias included factor V Leiden and prothrombin G20210A mutations, deficiency of protein S, protein C and antithrombin, antiphospholipid syndrome, and increased levels of factor VIII. The prevalence of thrombophilia was compared to healthy controls obtained from previously published data on thrombophilia distribution in cohorts of the Western European adult general population. Comparing PVT patients with healthy controls, significantly increased odds of presence of lupus anticoagulant (crude odds ratio (OR) 6.2, 95% confidence interval (CI) 1.8–20.6) were found, whereas no significantly increased odds of inherited thrombophilia were demonstrated. In conclusion, routine testing for inherited thrombophilia in PVT patients does not seem indicated. However, PVT patients should still be tested for antiphospholipid antibodies because patients meeting the criteria for antiphospholipid syndrome preferentially should receive vitamin K antagonists as anticoagulant therapy.

摘要

门静脉血栓形成 (PVT) 是一种罕见但严重的疾病。几个风险因素易患 PVT。然而，尚不清楚血栓形成倾向在何种程度上导致 PVT 风险以及 PVT 患者是否应常规转诊进行血栓形成倾向检测。本研究的目的是调查 PVT 患者血栓形成倾向的患病率，以阐明 PVT 患者血栓形成倾向检测的相关性。临床数据和血栓形成倾向的调查结果进行了系统的所有PVT患者获得简称中心血友病和血栓形成，奥胡斯大学医院，丹麦血栓形成倾向测试1间^日2010年1月和31^日2018年十二月（ñ = 93）。研究的血栓形成倾向包括因子 V Leiden 和凝血酶原 G20210A 突变、蛋白 S、蛋白 C 和抗凝血酶缺乏、抗磷脂综合征以及因子 VIII 水平升高。将血栓形成倾向的患病率与从先前公布的西欧成人一般人群队列中血栓形成倾向分布数据中获得的健康对照进行比较。将 PVT 患者与健康对照者进行比较，发现狼疮抗凝剂存在的几率显着增加（粗比值比 (OR) 6.2，95% 置信区间 (CI) 1.8-20.6），而未证明遗传性血栓形成倾向的几率显着增加。总之，对 PVT 患者遗传性血栓形成倾向的常规检测似乎没有指征。然而，