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产后获得性甲型血友病的表现为筋膜切开术后出现危及生命的出血的前臂骨筋膜室综合征:一例报告

Compartment syndrome of the forearm with life-threatening bleeding after fasciotomy as the presenting sign of postpartum acquired hemophilia A: a case report.

作者信息

Van Laer Marie, Penaloza Andrea, Stockman Willem, Meert Philippe, Lambert Catherine, Hermans Cédric

机构信息

Division of Emergency Medicine.

Division of Critical Care Medicine, AZ Delta Hospital, Roeselare, Belgium.

出版信息

Blood Coagul Fibrinolysis. 2019 Apr;30(3):120-126. doi: 10.1097/MBC.0000000000000799.

DOI:10.1097/MBC.0000000000000799
PMID:30864964
Abstract

: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the development of autoantibodies against clotting factor VIII. Although the cause of this disorder remains obscure, it is often linked to malignancies, drug administration, autoimmune diseases and pregnancy. In pregnancy-associated AHA, hemorrhagic symptoms usually present 1-4 months peripartum, however they may occur up to 1-year postpartum. Compartment syndrome of the forearm is also very uncommon complication of AHA but can have devastating consequences. We report a rare case of a compartment syndrome of the forearm in a 30-year-old woman 2.5 months postpartum as the presentation of pregnancy-associated AHA.

摘要

获得性血友病A(AHA)是一种罕见的出血性疾病,由针对凝血因子VIII的自身抗体产生所致。尽管这种疾病的病因尚不清楚,但通常与恶性肿瘤、药物使用、自身免疫性疾病和妊娠有关。在妊娠相关的AHA中,出血症状通常在围产期1 - 4个月出现,然而也可能在产后长达1年时发生。前臂骨筋膜室综合征也是AHA非常罕见的并发症,但可能产生严重后果。我们报告一例罕见病例,一名30岁女性在产后2.5个月出现前臂骨筋膜室综合征,作为妊娠相关AHA的表现。

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1
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引用本文的文献

1
Acquired factor VIII deficiency in a nulliparous patient undergoing induction of labor.一位未生育过的产妇在引产过程中获得性因子VIII缺乏。
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Fasciotomy on a female patient with acquired factor VIII: A case report and literature review of management.一名获得性因子 VIII 缺乏女性患者的筋膜切开术:病例报告及治疗文献综述
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Acute forearm compartment syndrome in the setting of acquired hemophilia A.
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