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一名产后患者以出血素质为表现的获得性血友病A:诊断与管理

Acquired hemophilia a presenting as a bleeding diathesis in a postpartum patient: diagnosis and management.

作者信息

Scully Mary-Frances, Shublaq Wael, Oliver Gillian D

机构信息

Memorial University of Newfoundland, Health Science Centre, St. John's, NF, Canada.

出版信息

J Obstet Gynaecol Can. 2002 May;24(5):430-2. doi: 10.1016/s1701-2163(16)30407-8.

Abstract

Antibodies against Factor VIII (FVIII) can develop in non-hemophiliac patients, causing the rare condition of acquired hemophilia. In 7.3% of the patients, the FVIII inhibitors appear either during pregnancy or in the postpartum period. In this case report, we present a non-hemophiliac patient, who presented five months postpartum with intermittent heavy vaginal bleeding, easy bruising, hemarthrosis, and recurrent frank hematuria. The woman presented to the emergency room with hematuria. Coagulation screening tests showed a prolonged APTT. Using a standard diagnostic algorithm, a Factor VIII inhibitor was detected. The treatment, cause of disease, and prognosis of this woman is presented in this paper as well as a literature review of acquired hemophilia A associated with pregnancy.

摘要

非血友病患者体内可产生抗凝血因子 VIII(FVIII)抗体,导致罕见的获得性血友病。7.3%的患者中,FVIII 抑制剂在孕期或产后出现。在本病例报告中,我们介绍了一名非血友病患者,她在产后五个月出现间歇性大量阴道出血、易瘀伤、关节积血和反复肉眼血尿。该名女性因血尿就诊于急诊室。凝血筛查试验显示活化部分凝血活酶时间(APTT)延长。采用标准诊断算法,检测到了 FVIII 抑制剂。本文介绍了该女性的治疗、病因及预后,并对与妊娠相关的获得性血友病 A 进行了文献综述。

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