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由于凝血因子VIII升高导致广泛的灾难性血栓形成。

Extensive catastrophic thromboses from elevation of factor VIII.

作者信息

Kropf Jacqueline, Cheyney Sarah, Vachon Josselin, Flaherty Philip, Vo Mai, Carlan Steve J

机构信息

Department of Internal Medicine.

Division of Critical Care Medicine.

出版信息

Clin Pract. 2020 Sep 4;10(3):1265. doi: 10.4081/cp.2020.1265.

Abstract

Catastrophic thrombotic syndrome, otherwise known as thrombotic storm (TS) is an extreme prothrombotic clinical syndrome that presents as rapid onset of multiple thromboembolic events affecting a large variety of vasculature. In recent studies, there has been a correlation of high plasma levels of factor VIII with thrombotic events. We present the case of a young man who exhibited multi-organ failure due to thrombotic storm. A 38-year-old male presented to the emergency department for progressive dyspnea and was diagnosed to have pulmonary embolism. The patient developed respiratory distress requiring intubation and was diagnosed with both an ST-elevation myocardial infarction and right cerebral infarction during the hospital course. The patient expired and autopsy revealed the cause of death to be myocardial, cerebral and renal infarction from widespread vascular thrombosis. Autopsy revealed cause of death to be elevated factor VIII associated thrombotic coagulopathy. Factor VIII level upon autopsy was 375% (55-200%). Although TS is rare, it can be lifethreatening if not recognized early. Survival depends on the prompt initiation and duration of anticoagulation.

摘要

灾难性血栓形成综合征,又称血栓风暴(TS),是一种极端的促血栓形成临床综合征,表现为多种血栓栓塞事件迅速发作,累及多种血管系统。在最近的研究中,血浆VIII因子水平升高与血栓形成事件之间存在相关性。我们报告一例因血栓风暴导致多器官衰竭的年轻男性病例。一名38岁男性因进行性呼吸困难就诊于急诊科,被诊断为肺栓塞。患者出现呼吸窘迫需要插管,并在住院期间被诊断为ST段抬高型心肌梗死和右脑梗死。患者死亡,尸检显示死因是广泛血管血栓形成导致的心肌、脑和肾梗死。尸检显示死因是VIII因子水平升高相关的血栓性凝血病。尸检时VIII因子水平为375%(55 - 200%)。虽然TS很少见,但如果不及早识别,可能会危及生命。生存取决于抗凝治疗的及时启动和持续时间。

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