| 摘要: |
| ［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2014-07～2016-04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1∶3，均未找到明确相关病因，首发症状均为皮肤软组织出血，所有患者均有活化的部分凝血活酶时间（APTT）延长，抗凝血因子Ⅷ（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是当前清除抗体的核心治疗方案。 |
| 关键词: 获得性血友病A 抗凝血因子Ⅷ抗体 旁路治疗 免疫抑制 |
| DOI：10.3969/j.issn.1674-3806.2017.07.19 |
| 分类号:R 55 |
| 基金项目: |
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| Acquired hemophilia A: a report of 4 cases and literature review |
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WANG Hui-ping, CHEN Yi, CHENG Peng, et al.
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Department of Hematology, the People′s Hospital of Guiping City, Guangxi 537200, China
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| Abstract: |
| ［Abstract］ Objective To improve the ability of diagnosing and treating acquired hemophilia A(AHA).Methods The clinical data of 4 patients with AHA who were diagnosed and treated in the First Affiliated Hospital of Guangxi Medical University and No.303 Hospital of Chinese People′s Liberation Army from July 2014 to April 2016 were retrospectively analyzed and the related literature was reviewed.Results The 4 patients were 44 to 73 years old, and the incidence ratio of male to female was 1∶3. There were no definite causes for the patients. The first clinical symptom of the patients was mucocutaneous hemorrhage or muscular hematoma. All the patients had isolated prolonged APTT, low level of FⅧ(0.4%～0.9%) and positive antibody titer(10.12～115 BU/ml). After the treatment with corticosteroid alone or combined with cyclophosphamide, the antibody titers in 4 patients were decreased and the FⅧ level increased. One case died of intracrantial hemorrhage.Conclusion (1)AHA is a rare bleeding disease which occurs predominantly in elderly patients.(2)Hemorrhage often occurs spontaneously,and the most frequent symptom is mucocutaneous hemorrhage or muscular hematoma.(3)Bypass treatment is recommended as the optimal antihemorrhagic treatment. Corticosteroid alone or combined with cyclophosphamide is still considered as the main treatment to eradicate the inhibitors. |
| Key words: Acquired hemophilia A(AHA) Inhibitors to factor Ⅷ Bypass treatment Immunosuppression agents |
