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。APTT98.6s，PT45.6s，TT24.6s,Fg2.6g/L,FⅧ:C2.5%,FⅧ:Ag3.0%,FⅨ:C88.O%,FX:C89.326。