【摘要】：目的：提高對獲得性血友病A的認識及診治水平。方法：對2014年7月至2016年4月廣西醫(yī)科大學第一附屬醫(yī)院及中國人民解放軍第三0三醫(yī)院確診的4例獲得性血友病A的臨床表現(xiàn)、實驗室檢查、治療及預后進行分析,并結(jié)合相關文獻進行復習并討論。結(jié)果：4例獲得性血友病A患者的發(fā)病年齡為44-73歲,男女發(fā)病比例為1：3,均未找到明確相關疾病,首發(fā)癥狀均為皮膚軟組織出血,所有患者均有APTT延長,FVⅢ活性下降(0.4-0.9%),FVⅢ抗體檢測陽性(10.12-115 BU/ml)。通過糖皮質(zhì)激素單用或聯(lián)合環(huán)磷酰胺治療后,4例患者的FVⅢ抗體滴度均較前下降,FVⅢ水平較前升高。1例患者在治療間期因腦出血死亡。結(jié)論：1.獲得性血友病A發(fā)病罕見,多見于老年人。2.臨床表現(xiàn)以自發(fā)性出血為主,其中以皮膚黏膜及肌肉軟組織出血多見。3.旁路治療為止血治療的一線方案,糖皮質(zhì)激素單用或聯(lián)合環(huán)磷酰胺仍然是當前清除抗體的核心治療方案。
[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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