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产后以进行性腹腔内出血、肌肉出血和血胸为表现的获得性甲型血友病:一例报告及文献复习

Acquired hemophilia A presenting as progressive intra-abdominal hemorrhage, muscle hemorrhage and hemothorax postpartum: A case report and literature review.

作者信息

Xu Liying, Chen Junfa, Zhou Xiajuan, Wu Lijuan, Tong Yixin, Zhu Ni, Huang Xiaomin, Zhang Zhuoyi

机构信息

Department of Emergency, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, Zhejiang 310006, P.R. China.

Department of Hematology, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, Zhejiang 310006, P.R. China.

出版信息

Exp Ther Med. 2019 Jan;17(1):633-638. doi: 10.3892/etm.2018.7031. Epub 2018 Nov 29.

Abstract

Acquired hemophilia A (AHA) is a rare antibody-mediated condition in which autoantibodies form against a coagulation factor, most commonly factor VIII (FVIII), causing severe coagulopathy. Here the present report presents a case of AHA in a 35-year-old postpartum woman with continuous polyserous bloody effusions who was admitted to the First Affiliated Hospital of Zhejiang Chinese Medical University (Hangzhou, China) in October 2017 without a history of trauma, anticoagulation treatment or coagulopathy. At presentation, the patient's hemoglobin level was low (70 g/l; normal range: 115-150 g/l) g/l, blood pressure was 89/58 mmHg (normal range, 90-140/60-90 mmHg), and activated partial thromboplastin time was 68.4 sec (normal range: 25.0-36.0 sec), with a normal international normalized ratio (0.94; normal range, 0.8-1.2). The reaction time in thrombography was prolonged (35.8 min; normal range: 5-10 min), coagulation FVIII had markedly decreased activity (12.6%; normal range, 60-150%), and FVIII inhibitor had a high titer [7.4 Bethesda units (BU)/ml; normal range, 0-0.6 BU/ml]. Notably, the patient's autoantibody level was markedly higher than normal (1:320; normal range: <1:100). The patient was successfully treated with bleeding control, eradication of FVIII inhibitor, and treatment of the underlying disease. To the best of our knowledge, this is the first case of AHA with polyserous bloody effusions in a patient with an autoimmune disorder during the postpartum period. Reports of such rare cases will aid the characterization of disease pathogenesis, which may in turn lead to the recognition and timely treatment of this rare disorder.

摘要

获得性血友病A(AHA)是一种罕见的抗体介导疾病,其中自身抗体针对凝血因子形成,最常见的是因子VIII(FVIII),导致严重的凝血病。本文报告了一例2017年10月入住浙江中医药大学附属第一医院(中国杭州)的35岁产后妇女AHA病例,该患者有持续性多浆膜腔血性积液,无创伤、抗凝治疗或凝血病病史。就诊时,患者血红蛋白水平低(70 g/l;正常范围:115 - 150 g/l),血压为89/58 mmHg(正常范围,90 - 140/60 - 90 mmHg),活化部分凝血活酶时间为68.4秒(正常范围:25.0 - 36.0秒),国际标准化比值正常(0.94;正常范围,0.8 - 1.2)。血栓弹力图反应时间延长(35.8分钟;正常范围:5 - 10分钟),凝血因子FVIII活性显著降低(12.6%;正常范围,60 - 150%),FVIII抑制物滴度高[7.4贝塞斯达单位(BU)/ml;正常范围,0 - 0.6 BU/ml]。值得注意的是,患者自身抗体水平明显高于正常(1:320;正常范围:<1:100)。该患者通过控制出血、消除FVIII抑制物及治疗基础疾病获得成功治疗。据我们所知,这是产后自身免疫性疾病患者中首例伴有多浆膜腔血性积液的AHA病例。此类罕见病例的报告将有助于疾病发病机制的特征描述,这反过来可能有助于识别和及时治疗这种罕见疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c8f/6307365/0ef75a0fd01d/etm-17-01-0633-g00.jpg

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