2018 年第 8 期 第 13 卷

汉族无诱因肺血栓栓塞症患者天然抗凝蛋白缺陷的发生率及临床特点分析

Prevalence and clinical characteristics of natural anticoagulant deficiency in Chinese Han patients with unprovoked pulmonary thromboembolism

作者：王增智赵秀清李杰高杨张向峰刘双

英文作者：

单位：100029首都医科大学附属北京安贞医院北京市心肺血管疾病研究所呼吸与危重症医学科（王增智、李杰、高杨、张向峰、刘双），检验科（赵秀清）

英文单位：

关键词：无诱因肺血栓栓塞症；遗传性易栓症；天然抗凝蛋白

英文关键词：

• 摘要：

• 【摘要】目的    探讨汉族无诱因肺血栓栓塞症（uPTE）患者中蛋白C、蛋白S和抗凝血酶Ⅲ 3种天然抗凝蛋白（NA）活性缺陷的发生率及临床特点。方法    回顾性选取2010年1月至2017年12月于首都医科大学附属北京安贞医院呼吸与危重症医学科住院的汉族uPTE患者265例，检测蛋白C、蛋白S和抗凝血酶Ⅲ活性并计算缺陷发生率。根据3种NA是否缺陷分为NA缺陷组和NA正常组，比较2组临床资料。结果    265例汉族uPTE患者3种NA缺陷总发生率为34.3%（91例），蛋白C和蛋白S复合缺陷（31例）、单纯蛋白S缺陷（28例）和单纯蛋白C缺陷（22例）居于发生率的前3位，总复合缺陷发生率为13.6%（36/265）。NA缺陷组年龄、肺血栓栓塞症初发年龄、临床出现呼吸困难比例小于/低于NA正常组［（55±13）岁比（64±11）岁、（50±14）岁比（63±12）岁、40.7%（37/91）比56.3%（98/174）］，有静脉血栓家族史比例高于NA正常组［23.1%（21/91）比3.4%（6/174）］，差异均有统计学意义（均P＜0.05）。结论    我国汉族uPTE患者中NA缺陷发生率高，携带NA缺陷的uPTE患者年龄和肺血栓栓塞症初发年龄较小、临床出现呼吸困难比例较低、有静脉血栓家族史比例较高。

• Objective    To investigate the prevalence rate of deficiency of natural anticoagulants(NA) including protein C, protein S and antithrombin Ⅲ in Chinese Han patients with unprovoked pulmonary thromboembolism (uPTE) and to analyze the clinical characteristics of NA deficiency. Methods    A total of 265 Chinese Han patients with uPTE who visited Beijing Anzhen Hospital, Capital Medical University from January 2010 to December 2017 were retrospectively analyzed. Expressions of protein C, protein S and antithrombin Ⅲ were detected. Clinical data were analyzed between patients with and without NA deficiency. Results    Prevalence rate of NA deficiency in 265 Chinese Han uPTE patients was 34.3%(n＝91). Thirty-one patients had protein C plus protein S deficiency; 28 patients had simple protein S deficiency; 22 patients had simple protein C deficiency; which were the top 3 common deficiency patterns. Combined NA deficiency rate was 13.6%(36/265). Age, onset age of pulmonary thromboembolism and incidence of dyspnea in patients with NA deficiency were significantly lower than those in patients without NA deficiency［（55±13）years vs （64±11）years, （50±14）years vs （63±12）years, 40.7%（37/91） vs 56.3%（98/174）］; family history of venous thrombosis ratio in patients with NA deficiency was significantly higher than that in patients without NA deficiency［23.1%（21/91） vs 3.4%（6/174）］（P＜0.05）. Conclusions    Prevalence rate of NA deficiency is high in Chinese Han patients with uPTE. Patients with NA deficiency may suffer from uPTE at a young age; dyspnea is uncommon; family history of venous thrombosis may increase the risk of disease.