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[一名多发伤患者获得性凝血因子V抑制剂]

[Acquired factor V inhibitors in a polytraumatized patient].

作者信息

Tukić Ljiljana, Stamatović Dragana, Tarabar Olivera, Elez Marija, Zorić Miodrag, Mandić-Radić Slavka

机构信息

Vojnomedicinska akademija, Klinika za hematologiju, Beograd, Srbija i Crna Gora.

出版信息

Vojnosanit Pregl. 2005 Jul-Aug;62(7-8):587-90. doi: 10.2298/vsp0508587t.

Abstract

BACKGROUND

Factor V (FV) inhibitors are a rare disorder reported for the first time about fifty years ago, mostly with the unknown cause. The appearance of FV inhibitors is usually preceded by surgery, infections, administration of antibiotics or transfusions. Clinical manifestations of the presence of FV inhibitors vary from mild to severe and in some instances fatal hemorrhage.

CASE REPORT

A healthy 51-year-old man with severe multiple injuries (traffic accident), and hemorrhage, which ocurred during the orthopedic treatment, was admitted with hemoptysis, epistaxis and hematoma of the right upper leg, and with prolonged prothrombin time (PT), and activated partial thromboplastin time (aPTT). Treatment with vitamin K, fresh-frozen plasma and cryoprecipitate stopped the hemorrhage, but the results of coagulation tests were not normalized. The correction of aPTT and PT with normal plasma showed the decreased activity of FV (1%) due to the presence of inhibitors (titer 17.5 IU). The abnormal resuts of coagulation tests remained for three weeks, but without clinically manifested hemorrhagic syndrome. At the fourth week after the appearance of FV inhibitors PT, aPTT and the activity of FV became normal and antibodies disappeared spontaneously.

CONCLUSION

Our patient with polytrauma developed a mild hemorrhagic syndrome due to the presence of FV inhibitors five weeks after the accident. Hemorrhage was treated with substitution therapy. The cause of the development of FV inhibitors was unclear ("fibrin glue" was not used during the orthopedic treatment). Factor V inhibitors disappeared spontaneously within four weeks. The fast spontaneous disappearance of FV inhibitors in our patient, confirmed the observations of some authors that they disappeared faster in those patients who were surgically treated prior to their appearance.

摘要

背景

凝血因子V(FV)抑制剂是一种罕见的疾病,大约在五十年前首次报道,其病因大多不明。FV抑制剂的出现通常先于手术、感染、抗生素使用或输血。FV抑制剂存在的临床表现从轻度到重度不等,在某些情况下会导致致命性出血。

病例报告

一名51岁健康男性,因严重多发伤(交通事故)并在骨科治疗期间发生出血入院,伴有咯血、鼻出血和右上腿血肿,凝血酶原时间(PT)延长,活化部分凝血活酶时间(aPTT)延长。使用维生素K、新鲜冰冻血浆和冷沉淀治疗后出血停止,但凝血试验结果未恢复正常。用正常血浆纠正aPTT和PT显示由于抑制剂的存在(滴度17.5 IU),FV活性降低(1%)。凝血试验异常结果持续了三周,但无临床表现的出血综合征。在FV抑制剂出现后的第四周,PT、aPTT和FV活性恢复正常,抗体自发消失。

结论

我们的多发伤患者在事故发生五周后因FV抑制剂的存在出现了轻度出血综合征。出血采用替代疗法治疗。FV抑制剂产生的原因尚不清楚(骨科治疗期间未使用“纤维蛋白胶”)。FV抑制剂在四周内自发消失。我们患者中FV抑制剂的快速自发消失,证实了一些作者的观察结果,即它们在出现前接受手术治疗的患者中消失得更快。

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