患者,男,36岁,自幼肌肉关节出血,牙龈出血,皮肤瘀斑;膝关节肿大,活动受限。家族史阳性。APTT 98.6s,PT 45.6s,TT24.6s,Fg2.6g/L,FⅧ:C 2.5%,FⅧ:Ag3.0%,FⅨ:C 88.0%,FⅩ:C 89.3%。