我室观察到孪生兄妹均为血友病的患者,这种情况比较少见,尚未见文献报道。现报告如下。病例报告孪生兄,10岁,血型A,生后至今常因轻外伤鼻衄、血肿,出血不止,关节畸型等,而皮肤粘膜未见出血点及紫癜。孪生妹,10岁,血型O。出血症状比孪生兄轻,轻外伤后仅有皮肤青紫,偶有出血不止。患者父母无血缘关系,其父在青年时常有鼻衄,而其母皮肤常有青紫斑,月经量多。其弟(7岁)也有齿龈出血史。讨论分析孪生兄凝血酶原时间正常,白陶土部分凝血活酶时间延长,凝血酶原消耗不佳,加红细胞素<180秒,提示内凝系统复合物Ⅰ障碍。Biggs 凝血活酶生成异常,能被正常硫酸钡吸附血浆纠正,而不能被正常血清所纠正,故为因子Ⅷ缺乏症,活动度16.5%,属轻型。根据出血时间正常,阿斯匹林耐量试验正常,血小板粘附指数正常可除外血管性假血友病。孪生妹凝血酶原时间、血清凝血酶原时间、白陶土部分凝 My room observed twins and sisters are hemophilia patients, this situation is relatively rare, has not been reported in the literature. The report is as follows. Case report twin brother, 10 years old, blood type A, often due to post-natal traumatic epistaxis, hematoma, bleeding more than, joint deformity, etc., and no skin and mucous membrane bleeding and purpura. Twin sister, 10 years old, blood type O. Bleeding symptoms than twin brother light, bruised skin after light trauma, occasional bleeding more than. Patients with no blood relationship between parents, their father often have epistaxis in youth, and its mother’s skin often have purple spots, menstrual flow and more. His brother (7 years old) also had bleeding gums. Discussion and analysis Twins brother prothrombin time is normal, kaolin part of the thromboplastin time, prothrombin consumption is not good, add erythrocyte <180 seconds, suggesting that the coagulation system complex I disorder. Biggs thromboplastin abnormalities, can be normal plasma barium sulfate adsorption corrected, but can not be corrected by normal serum, it is factor â ... § deficiency, activity 16.5%, is light. According to the normal bleeding time, normal aspirin tolerance test, platelet adhesion index can be normal except for von Willebrand disease. Twin sister prothrombin time, serum prothrombin time, part of the white clay coagulation