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围产期获得性血友病A:诊断与治疗困境

Acquired Hemophilia A in Peripartum Period: Diagnostic and Therapeutic Dilemma.

作者信息

Bin Waqar Syed Hamza, Khoury Leen, Hussain Ather, McFarlane Isabel M

机构信息

Hematology and Oncology, SUNY Downstate Medical Center, New York, USA.

Hematology and Oncology, Rochester General Hospital, Rochester, USA.

出版信息

Cureus. 2021 Aug 1;13(8):e16803. doi: 10.7759/cureus.16803. eCollection 2021 Aug.

DOI:10.7759/cureus.16803
PMID:34513409
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8406369/
Abstract

Acquired hemophilia A (AHA) is a bleeding diathesis caused by auto-antibody generation against factor VIII, an essential component of the coagulation cascade. Although having many etiologies, pregnancy is also one of the conditions associated with AHA. It mostly presents as a raised activated partial thromboplastin time (aPTT), and during the peripartum and postpartum period, concern for AHA should be raised as delays in diagnosis can be detrimental. Herein, we present a case of a 31-year-old female with sickle cell trait who developed venous bleeding and, later, neuraxial, musculoskeletal, and subcutaneous bleeding. She underwent an extensive course of treatment before getting into remission.

摘要

获得性血友病A(AHA)是一种由于针对凝血级联反应的重要组成部分——凝血因子VIII产生自身抗体而导致的出血性素质。尽管病因众多,但妊娠也是与AHA相关的情况之一。它主要表现为活化部分凝血活酶时间(aPTT)升高,在围产期和产后期间,应提高对AHA的警惕,因为诊断延迟可能有害。在此,我们报告一例31岁具有镰状细胞性状的女性病例,该患者出现静脉出血,随后出现神经轴、肌肉骨骼和皮下出血。她在病情缓解前接受了广泛的治疗过程。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f56d/8406369/e1a367146fb3/cureus-0013-00000016803-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f56d/8406369/673dd4c3ae6c/cureus-0013-00000016803-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f56d/8406369/00f2cfd27b5e/cureus-0013-00000016803-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f56d/8406369/30ee730329b8/cureus-0013-00000016803-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f56d/8406369/e1a367146fb3/cureus-0013-00000016803-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f56d/8406369/673dd4c3ae6c/cureus-0013-00000016803-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f56d/8406369/00f2cfd27b5e/cureus-0013-00000016803-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f56d/8406369/30ee730329b8/cureus-0013-00000016803-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f56d/8406369/e1a367146fb3/cureus-0013-00000016803-i04.jpg

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本文引用的文献

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Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report.以腹腔积血为表现的产后获得性甲型血友病:一例报告
Cureus. 2020 Dec 1;12(12):e11817. doi: 10.7759/cureus.11817.
2
Treatment of acquired hemophilia A, a balancing act: results from a 27-year Dutch cohort study.获得性血友病 A 的治疗:一种平衡的艺术——来自一项 27 年荷兰队列研究的结果。
Am J Hematol. 2021 Jan;96(1):51-59. doi: 10.1002/ajh.26009. Epub 2020 Oct 10.
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Pregnancy-related acquired hemophilia A initially manifesting as pleural hemorrhage: A case report.
美国非血友病专家对获得性血友病A有效止血管理潜在障碍的综述
Cureus. 2023 Jan 18;15(1):e33927. doi: 10.7759/cureus.33927. eCollection 2023 Jan.
妊娠相关获得性甲型血友病最初表现为胸腔出血:一例报告
Medicine (Baltimore). 2019 Jan;98(3):e14119. doi: 10.1097/MD.0000000000014119.
4
Newly Diagnosed Acquired Hemophilia A Manifesting as Massive Intracranial Hemorrhage Following a Neurosurgical Procedure.新诊断的获得性甲型血友病在神经外科手术后表现为大量颅内出血。
World Neurosurg. 2018 Mar;111:175-180. doi: 10.1016/j.wneu.2017.12.016. Epub 2017 Dec 15.
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Acquired hemophilia A: Updated review of evidence and treatment guidance.获得性血友病 A:证据和治疗指南的最新综述。
Am J Hematol. 2017 Jul;92(7):695-705. doi: 10.1002/ajh.24777. Epub 2017 Jun 5.
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The factor VIII protein and its function.凝血因子 VIII 蛋白及其功能。
Acta Biochim Pol. 2016;63(1):11-16. doi: 10.18388/abp.2015_1056. Epub 2016 Jan 28.
7
Definitions in hemophilia: communication from the SSC of the ISTH.血友病的定义:来自国际血栓与止血学会(ISTH)科学标准化委员会(SSC)的交流文件
J Thromb Haemost. 2014 Nov;12(11):1935-9. doi: 10.1111/jth.12672. Epub 2014 Sep 3.
8
Pre-analytical heat treatment and a FVIII ELISA improve Factor VIII antibody detection in acquired haemophilia A.分析前热处理和FVIII ELISA可提高获得性血友病A中VIII因子抗体的检测率。
Br J Haematol. 2014 Sep;166(6):953-6. doi: 10.1111/bjh.12923. Epub 2014 May 9.
9
Outcome of acquired haemophilia in France: the prospective SACHA (Surveillance des Auto antiCorps au cours de l'Hémophilie Acquise) registry.法国获得性血友病的结局:前瞻性 SACHA(获得性血友病治疗过程中自身抗体监测)登记研究。
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Blood. 2012 Jul 5;120(1):39-46. doi: 10.1182/blood-2012-02-408930. Epub 2012 May 22.