第ⅩⅢ因子缺乏症,首为Duckert等所报告(1960)。本症分先天性和后天性两种,少数患者虽可大量出血,以至死亡,但进行一般凝血检查却均属正常,故临床上诊断较为困难。近年来对第ⅩⅢ因子的作用机理逐渐明确,实验室检查技术不断进步,本症的发现率也有所提高。现将有关材料综述如下。 Factor XIII deficiency, first reported by Duckert et al. (1960). The syndrome of congenital and acquired two kinds, although a small number of patients may be a large number of bleeding, and even death, but the general coagulation tests are normal, so the clinical diagnosis is more difficult. In recent years, the mechanism of action of factor XIII gradually clear, laboratory testing technology continues to progress, the detection rate of this disease has also been increased. The relevant materials are summarized below.