Institute of Hematology, Lady Davis Carmel Medical Center, Haifa, Israel.
Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel; and.
Blood. 2017 Mar 2;129(9):1210-1215. doi: 10.1182/blood-2016-09-742262. Epub 2016 Dec 30.
Factor XI deficiency is one of the rare inherited coagulation factor deficiencies. However, its incidence is high within the Ashkenazi Jewish community. Because factor XI displays both procoagulant and antifibrinolytic activities, it has been postulated that an underlying cardiovascular benefit may exist with factor XI deficiency. This historical cohort study was performed using the electronic database of Clalit Health Services, the largest health care provider in Israel. All adults tested for factor XI activity between 2002 and 2014 were included in the study. Factor XI activity was classified into 3 categories: normal (activity >50%), mild deficiency (activity = 30%-50%), and moderate-severe deficiency (activity ≤30%). The cohort was followed until 31 December 2015 for incidence of cardiovascular events (composite of myocardial infarction, stroke, and transient ischemic attack) and venous thromboembolism (VTE). Of the 10 193 included patients, 8958 (88.9%) had normal factor XI activity, 690 (6.8%) had mild deficiency, and 542 (5.3%) had moderate-severe deficiency. Compared with individuals with normal activity, the adjusted hazard ratio (HR) for cardiovascular events was 0.52 (95% confidence interval [CI], 0.31-0.87) in those with mild deficiency, and 0.57 (95% CI, 0.35-0.93) in those with moderate-severe factor XI deficiency. The incidence of VTE was lower in those with factor XI deficiency (activity <50%) compared with those with normal activity; adjusted HR = 0.26 (95% CI, 0.08-0.84). In summary, factor XI deficiency is associated with decreased incidence of cardiovascular events and VTE.
XI 因子缺乏症是一种罕见的遗传性凝血因子缺乏症。然而，在阿什肯纳兹犹太人群体中，其发病率很高。由于 XI 因子同时具有促凝和抗纤维蛋白溶解活性，因此有人推测 XI 因子缺乏可能存在潜在的心血管益处。这项历史性队列研究是使用 Clalit 健康服务的电子数据库进行的，Clalit 健康服务是以色列最大的医疗保健提供商。所有在 2002 年至 2014 年间接受 XI 因子活性检测的成年人都被纳入了这项研究。XI 因子活性分为 3 类：正常（活性>50%）、轻度缺乏（活性=30%-50%）和中重度缺乏（活性≤30%）。该队列一直随访至 2015 年 12 月 31 日，以记录心血管事件（心肌梗死、中风和短暂性脑缺血发作的复合事件）和静脉血栓栓塞症（VTE）的发生率。在纳入的 10193 名患者中，8958 名（88.9%）具有正常的 XI 因子活性，690 名（6.8%）具有轻度缺乏，542 名（5.3%）具有中重度缺乏。与活性正常的个体相比，轻度缺乏的个体发生心血管事件的校正风险比（HR）为 0.52（95%置信区间 [CI]，0.31-0.87），中重度缺乏的个体为 0.57（95% CI，0.35-0.93）。与活性正常的个体相比，XI 因子缺乏症（活性<50%）患者的 VTE 发生率较低；校正 HR=0.26（95% CI，0.08-0.84）。总之，XI 因子缺乏与心血管事件和 VTE 的发生率降低有关。
