获得性血友病A：4例病例报道并文献复习

发布时间：2018-10-09 18:25

【摘要】：目的：提高对获得性血友病A的认识及诊治水平。方法：对2014年7月至2016年4月广西医科大学第一附属医院及中国人民解放军第三0三医院确诊的4例获得性血友病A的临床表现、实验室检查、治疗及预后进行分析,并结合相关文献进行复习并讨论。结果：4例获得性血友病A患者的发病年龄为44-73岁,男女发病比例为1：3,均未找到明确相关疾病,首发症状均为皮肤软组织出血,所有患者均有APTT延长,FVⅢ活性下降(0.4-0.9%),FVⅢ抗体检测阳性(10.12-115 BU/ml)。通过糖皮质激素单用或联合环磷酰胺治疗后,4例患者的FVⅢ抗体滴度均较前下降,FVⅢ水平较前升高。1例患者在治疗间期因脑出血死亡。结论：1.获得性血友病A发病罕见,多见于老年人。2.临床表现以自发性出血为主,其中以皮肤黏膜及肌肉软组织出血多见。3.旁路治疗为止血治疗的一线方案,糖皮质激素单用或联合环磷酰胺仍然是当前清除抗体的核心治疗方案。
[Abstract]:Objective: to improve the understanding, diagnosis and treatment of acquired hemophilia A. Methods: from July 2014 to April 2016, the clinical manifestations, laboratory examination, treatment and prognosis of 4 cases of acquired hemophilia A diagnosed in the first affiliated Hospital of Guangxi Medical University and the 303 Hospital of the Chinese people's Liberation Army were analyzed. Combined with the relevant literature review and discussion. Results the onset age of 4 cases of acquired hemophilia A was 44 to 73 years old, and the ratio of male to female was 1: 3. None of them found any related diseases. The first symptom was skin and soft tissue hemorrhage. All patients had decreased activity of APTT prolongation FV 鈪，

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