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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

Fasciotomy on a female patient with acquired factor VIII: A case report and literature review of management.

作者信息

Lovell Katie, Peters Bethlehem, Pasli Melisa, Kennedy Katie, Liles Darla, Pories Walter

机构信息

East Carolina University Brody School of Medicine Greenville North Carolina USA.

Department of Hematology/Oncology ECU Health Brody School of Medicine Greenville North Carolina USA.

出版信息

Clin Case Rep. 2023 Aug 3;11(8):e7773. doi: 10.1002/ccr3.7773. eCollection 2023 Aug.

DOI:10.1002/ccr3.7773
PMID:37546159
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10400796/
Abstract

KEY CLINICAL MESSAGE

Acquired factor VIII inhibitors can be a rare cause of extensive intramuscular bleeding requiring fasciotomy. The subsequent postoperative period requires close monitoring due to high risk of fatal blood loss.

ABSTRACT

Acquired factor VIII inhibitors are a rare cause of often extensive bleeding and subsequently large hematomas. This disorder's overall mortality can reach 38%, largely due to immunosuppression and subsequent infections or an underlying cause such as malignancy. The patient in this case study presented with a hematoma and extensive ecchymosis of the hand and forearm, which continued to progress, precipitating compartment syndrome of the hand and forearm and ultimately requiring fasciotomy. The combination of factors led to significant blood loss in the postoperative period requiring major fluid resuscitation and intensive care unit (ICU) level care. Due to this disorder's rarity and overall mortality, we present this case report with a literature review for management of acquired hemophilia in the setting of urgent fasciotomy.

摘要

关键临床信息

获得性凝血因子VIII抑制物可能是导致广泛肌肉内出血并需要进行筋膜切开术的罕见原因。由于存在致命失血的高风险,术后需要密切监测。

摘要

获得性凝血因子VIII抑制物是导致经常出现广泛出血及随后形成大血肿的罕见原因。这种疾病的总体死亡率可达38%,主要是由于免疫抑制及随后的感染或潜在病因(如恶性肿瘤)。本病例研究中的患者手部和前臂出现血肿及广泛瘀斑,并持续进展,引发手部和前臂骨筋膜室综合征,最终需要进行筋膜切开术。多种因素导致术后大量失血,需要大量液体复苏及重症监护病房(ICU)级别的护理。鉴于这种疾病的罕见性及总体死亡率,我们呈现此病例报告并进行文献综述,以探讨在紧急筋膜切开术情况下获得性血友病的管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eba1/10400796/84258baae56e/CCR3-11-e7773-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eba1/10400796/63be13921145/CCR3-11-e7773-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eba1/10400796/84258baae56e/CCR3-11-e7773-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eba1/10400796/63be13921145/CCR3-11-e7773-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eba1/10400796/84258baae56e/CCR3-11-e7773-g003.jpg

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Ann Med Surg (Lond). 2022 Aug;80:104275. doi: 10.1016/j.amsu.2022.104275. Epub 2022 Jul 31.
2
Acute forearm compartment syndrome in the setting of acquired hemophilia A.获得性血友病A患者出现的急性前臂骨筋膜室综合征。
Case Reports Plast Surg Hand Surg. 2022 May 13;9(1):140-144. doi: 10.1080/23320885.2022.2071274. eCollection 2022.
3
Forearm compartment syndrome due to acquired hemophilia that required massive blood transfusions after fasciotomy: A case report.
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World J Clin Cases. 2021 Nov 6;9(31):9592-9597. doi: 10.12998/wjcc.v9.i31.9592.
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Severe acquired haemophilia associated with asymptomatic SARS-CoV-2 infection.与无症状 SARS-CoV-2 感染相关的严重获得性血友病。
BMJ Case Rep. 2021 Jul 20;14(7):e242884. doi: 10.1136/bcr-2021-242884.
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New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome.获得性血友病和获得性血管性血友病综合征诊断与管理的新进展
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