Hilgartner M W
Haematologia (Budap). 1986;19(1):21-32.
The development of inhibitors against factor VIII or IX in hemophilic or nonhemophilic patients is a relatively rare condition, but basic and clinical studies of these inhibitors have been extremely fruitful and have led to the following: a better understanding of the structure-function relationships of the factor VIII and IX molecules; increased knowledge of the genetic defect in classic hemophilia and hemophilia B; newer insights into antibody formation against clotting factors; and an increased number of therapeutic options for the treatment of patients who have inhibitors to factor VIII or IX. The prevalence of inhibitors to factor VIII:C in the hemophiliac population of the U.S. was obtained in the NHLBI multi-center trial using 1500 patients and is estimated to be 14.5%. The vast majority of these inhibitors occurred in severely affected hemophiliacs less than twenty years of age with factor VIII:C levels of less than 0.03 u/ml. However, occasional older patients or those with F. VIII:C greater than 0.03 u/ml did develop inhibitors.
在血友病或非血友病患者中，针对凝血因子VIII或IX的抑制物产生是一种相对罕见的情况，但对这些抑制物的基础和临床研究成果极其丰硕，带来了以下成果：对凝血因子VIII和IX分子的结构-功能关系有了更好的理解；对经典血友病和B型血友病的基因缺陷有了更多认识；对针对凝血因子的抗体形成有了新的见解；以及为治疗对凝血因子VIII或IX有抑制物的患者增加了治疗选择。在美国国立心肺血液研究所（NHLBI）的多中心试验中，使用1500名患者得出了美国血友病患者群体中凝血因子VIII:C抑制物的患病率，估计为14.5%。这些抑制物绝大多数出现在年龄小于20岁、凝血因子VIII:C水平低于0.03u/ml的重度血友病患者中。然而，偶尔也有老年患者或凝血因子VIII:C大于0.03u/ml的患者会产生抑制物。
