31例获得性凝血因子缺乏症的回顾性分析

doi:10.6040/j.issn.1671-7554.0.2022.0803

摘要/Abstract

摘要： 目的探讨获得性凝血因子缺乏症的病因、临床表现、治疗、转归。方法回顾性分析山东大学齐鲁医院2011年7月至2021年10月确诊为获得性凝血因子缺乏症患者31例的临床资料。获得性血友病A(AHA)患者根据FⅧ抑制物水平分组并进行组间相关性分析。结果 31例获得性凝血因子缺乏症患者中,29例为AHA,2例为获得性凝血因子V缺乏症(AFVD)。AHA最常见临床表现为皮肤瘀斑(62.07%)和肌肉软组织血肿(34.48%),AFVD则分别表现为血尿和皮肤出血点。凝血因子Ⅷ(FⅧ)抑制物滴度高的患者活化的部分凝血活酶时间(APTT)更长,凝血因子Ⅷ活性(FⅧ: C )水平更低,差异有统计学意义(P<0.05),但FⅧ抑制物滴度高低与患者的出血严重程度无相关性。结论筛查患者病因,结合患者的病因、年龄、基础病及凝血因子水平等实验室检查评估后制定个性化治疗方案,对于获得性凝血因子缺乏症的管理具有重要意义。

关键词: 获得性血友病A, 凝血因子缺乏, 抑制物, 免疫抑制剂

Abstract: Objective To explore the etiology, clinical manifestations, treatment and outcomes of acquired coagulation factor deficiency. Methods Clinical data of 31 patients with acquired coagulation factor deficiency during Jul. 2011 and Oct. 2021 were retrospectively analyzed. Patients with acquired hemophilia A(AHA)were grouped according to FⅧ inhibitor levels and intergroup correlation analysis was conducted. Results Of the 31 cases, 29 were AHA and 2 were acquired coagulation factor V deficiency(AFVD). The most common clinical symptoms of AHA were subcutaneous bleeding(62.07%)and muscle and soft tissue hematoma(34.48%). The AFVD patients had symptoms of hematuria and skin hemorrhagic spots. Patients with higher FⅧ inhibitor titer had longer activated partial thromboplastin time and lower level of FⅧ: C(P<0.05), but there was no correlation between the level of FⅧ inhibitor titer and severity of bleeding. Conclusion Screening the etiology of patients, combined with the comprehensive evaluation of the etiology, age, underlying diseases, levels of coagulation factors and other indicators to formulate an individualized treatment plan, has an important guiding significance for the clinical management of acquired coagulation factor deficiency.

Key words: Acquired hemophilia A, Coagulation factor deficiency, Inhibitor, Immunosuppressive agents

中图分类号:

R552

尚阳丽,钟光彩,李世洁,孙洺山,陈春燕. 31例获得性凝血因子缺乏症的回顾性分析[J]. 山东大学学报 (医学版), 2023, 61(5): 25-30.

SHANG Yangli, ZHONG Guangcai, LI Shijie, SUN Mingshan, CHEN Chunyan. A retrospective analysis of 31 patients with acquired coagulation factor deficiency[J]. Journal of Shandong University (Health Sciences), 2023, 61(5): 25-30.

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