遗传性凝血因子Ⅶ缺乏是一种少见的凝血疾患,1951年Alexander等首先报导。Rogni等在1981年广泛复习文献时发现在东方人种之中尚无遗传性因子Ⅶ缺乏的报告。作者在一个中国家族中发现有遗传性因子Ⅶ缺乏。研究对象:包括先证者(系32岁中国女性)和她家族4代人中的29个成员。患者不是近亲结婚的后代。她的一个弟弟有出血倾向,于25岁死亡。家族中其他人无出血倾向。患者在儿童时期容易因挫伤而出血,经常有鼻衄及牙龈出血。月经量多,偶尔可长达2～3周。自然分娩三次,其中一次阴道大出血,输血200ml。16岁以后不论有无创伤史,常有关节积血而累及右肘和双膝关节,并导致关节活动受限。27岁时作关节固定术并发大出血,输血200ml。1978年8月因左膝关节疼痛肿胀而入院。住院中发现凝血酶原时间延长,故作全面的凝血试验 Hereditary coagulation factor Ⅶ deficiency is a rare coagulation disorders, Alexander first reported in 1951. In an extensive review of the literature in 1981, Rogni et al. Found no evidence of the absence of hereditary Ⅶ in Oriental populations. The authors found a hereditary factor Ⅶ lack in a Chinese family. Subjects: Including probands (32-year-old Chinese woman) and her family of 4 generations of 29 members. Patients are not offspring married offspring. One of her younger brothers had a bleeding tendency and died at the age of 25. Other people in the family have no bleeding tendency. Patients in childhood bleeding easily due to contusion, often nose and gingival bleeding. Menstrual volume, and occasionally up to 2 to 3 weeks. Natural childbirth three times, one of the vaginal bleeding, blood transfusions 200ml. After 16 years of age, regardless of whether there is a history of trauma, often involving hemarthrosis and right knee and knee joint, and lead to limited joint activity. 27 years old for joint surgery complicated with bleeding, blood transfusions 200ml. August 1978 due to swelling of the left knee joint pain and swelling. Prothrombin time was found to be prolonged in hospital and was a comprehensive coagulation test