获得性FⅩⅢ缺乏的研究进展

Advances in the study of acquired FⅩⅢ deficiency

血浆凝血因子ⅩⅢ(pFⅩⅢ)主要通过连接纤维蛋白分子和募集α2抗纤溶酶而强化和稳定血栓。该因子缺乏时引起“迟发性”为特征的出血和自发性流产。根据发病机制,分为先天性和获得性两种情况。获得性FⅩⅢ缺乏症罕见,也不容易被早期识别,可由获得性因子抑制物引起,也可以由导致FⅩⅢ合成减少或消耗增加的疾病引起。最常见的临床表现是软组织血肿,发生于70%以上的患者,中枢神经系统出血也相对常见。随着报道的获得性FⅩⅢ缺乏患者在逐渐增加,渐渐引起了人们的重视,但仍容易漏诊和诊断延迟,本文综述获得性FⅩⅢ缺乏机制、诊断和治疗的研究进展。

Plasma coagulation factor ⅩⅢ(pFⅩⅢ)strengthens and stabilizes thrombus mainly by linking fibrin molecules and recruitingα2 antiplasminase.Deficiency of this factor causes bleeding characterized by"late onset"and spontaneous abortion.According to the pathogenesis,it can be divided into two types:congenital and acquired.Acquired FⅩⅢdeficiency,which is rare and not easy to identify early,can be caused by an acquired factor inhibitor or by a disease that results in reduced FXⅢsynthesis or increased consumption.The most common clinical manifestation is soft tissue hematoma,which occurs in more than 70%of patients,and central nervous system bleeding is also relatively common.With the increasing number of patients with acquired FⅩⅢdeficiency reported,attention has been gradually drawn,but it is still easy to miss and delay diagnosis.This paper reviews the research progress on the mechanism,diagnosis and treatment of acquired FⅩⅢdeficiency.