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以腹腔积血为表现的产后获得性甲型血友病:一例报告

Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report.

作者信息

Azam Khalid, Batool Zainab, Malik Ayesha, Chaudhry Manahil, Abdullah Mohammad

机构信息

Medicine, Combined Military Hospital Lahore Medical College and Institute of Dentistry, Lahore, PAK.

Medicine, Hameed Latif Hospital, Lahore, PAK.

出版信息

Cureus. 2020 Dec 1;12(12):e11817. doi: 10.7759/cureus.11817.

DOI:10.7759/cureus.11817
PMID:33409062
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7781504/
Abstract

Acquired hemophilia A (AHA) is a bleeding diathesis caused by the production of autoantibodies to factor VIII (FVIII). It manifests as an isolated deranged activated partial thromboplastin time (aPTT) indicating a defect in the intrinsic coagulation pathway. Herein, we report a case of a 26-year-old woman who presented with hemoperitoneum in the postpartum period following a lower segment Caesarean section (LSCS). AHA carries significant mortality if it remains undiagnosed, and early recognition and measures to eradicate the acquired inhibitors are the mainstays of its management.

摘要

获得性血友病A(AHA)是一种由针对凝血因子VIII(FVIII)产生自身抗体引起的出血性素质。其表现为孤立的活化部分凝血活酶时间(aPTT)异常,提示内源性凝血途径存在缺陷。在此,我们报告一例26岁女性病例,该患者在低位剖宫产术后产褥期出现腹腔积血。如果AHA未被诊断,会有显著的死亡率,早期识别和消除获得性抑制物的措施是其治疗的主要支柱。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae3d/7781504/480ec2fb0965/cureus-0012-00000011817-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae3d/7781504/480ec2fb0965/cureus-0012-00000011817-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae3d/7781504/480ec2fb0965/cureus-0012-00000011817-i01.jpg

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本文引用的文献

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International recommendations on the diagnosis and treatment of acquired hemophilia A.国际上关于获得性血友病 A 的诊断和治疗建议。
Haematologica. 2020 Jul;105(7):1791-1801. doi: 10.3324/haematol.2019.230771. Epub 2020 May 7.
2
Pregnancy-related acquired hemophilia A initially manifesting as pleural hemorrhage: A case report.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.
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Acquired haemophilia A: a rare cause of postpartum haemorrhage.
一名患有先前未诊断出的结缔组织病的西班牙裔孕妇出现严重血尿,诊断为获得性因子VIII缺乏症。
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Acquired Hemophilia A in Peripartum Period: Diagnostic and Therapeutic Dilemma.围产期获得性血友病A:诊断与治疗困境
Cureus. 2021 Aug 1;13(8):e16803. doi: 10.7759/cureus.16803. eCollection 2021 Aug.
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Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry.妊娠相关性获得性血友病 A:来自欧洲获得性血友病(EACH2)登记处的结果。
BJOG. 2012 Nov;119(12):1529-37. doi: 10.1111/j.1471-0528.2012.03469.x. Epub 2012 Aug 20.
7
Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2).获得性血友病 A 的人口统计学和临床数据:来自欧洲获得性血友病登记处(EACH2)的结果。
J Thromb Haemost. 2012 Apr;10(4):622-31. doi: 10.1111/j.1538-7836.2012.04654.x.
8
Sequential thrombosis and bleeding in a woman with a prolonged activated partial thromboplastin time.一名女性的活化部分凝血活酶时间延长,出现连续的血栓形成和出血。
Thromb J. 2011 Oct 27;9:16. doi: 10.1186/1477-9560-9-16.
9
The first case of postpartum acquired hemophilia A in Korea.韩国首例产后获得性血友病 A 病例。
J Korean Med Sci. 2011 Sep;26(9):1247-9. doi: 10.3346/jkms.2011.26.9.1247. Epub 2011 Sep 1.
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Haemophilia. 2010 Jan;16(1):14-7. doi: 10.1111/j.1365-2516.2009.02049.x. Epub 2009 Jun 4.