Uemura Y, Niiya K, Takeuchi T, Miyoshi I
Department of Medicine, Kochi Medical School.
Rinsho Ketsueki. 1991 Jan;32(1):39-42.
A 29-year-old women, who had been treated by hemodialysis for 5 years because of chronic renal failure, developed bleeding tendency in March 1989. Laboratory data showed prolonged activated partial thromboplastin time which was not corrected by addition of normal plasma; factor VIII activity was less than 1% and factor VIII inhibitor 70 Bethesda units/ml. The inhibitor was eluted in the second peak which corresponded to IgG when the plasma was subjected to Sephacryl S 200 column. The further purified IgG fraction by passing through protein A column showed a factor VIII inhibitor activity of 52 Bethesda units/ml. The factor VIII inhibitor epitopes were examined by western blotting technique using factor VIII purified by monoclonal antibody as the antigen. The factor VIII preparation used was composed of a doublet of light chain (Mr 80,000) and three heavy chains (Mr 160,000-200,000) when examined by immunoblotting using anti-factor VIII light and heavy chains monoclonal antibodies after SDS-PAGE. Factor VIII inhibitor that arose in a hemophilia A patient recognized the light chain, and the inhibitor in this case reacted to the heavy chain of factor VIII.
一名29岁女性因慢性肾衰竭接受血液透析治疗5年，于1989年3月出现出血倾向。实验室检查显示活化部分凝血活酶时间延长，加入正常血浆后未得到纠正；因子VIII活性低于1%，因子VIII抑制物为70贝塞斯达单位/毫升。当血浆通过Sephacryl S 200柱时，抑制物在与IgG相对应的第二个峰中被洗脱。通过蛋白A柱进一步纯化的IgG组分显示因子VIII抑制物活性为52贝塞斯达单位/毫升。采用经单克隆抗体纯化的因子VIII作为抗原，通过蛋白质印迹技术检测因子VIII抑制物表位。经SDS-PAGE后，使用抗因子VIII轻链和重链单克隆抗体进行免疫印迹检测，所用的因子VIII制剂由一条轻链（分子量80,000）和三条重链（分子量160,000 - 200,000）的双峰组成。一名甲型血友病患者产生的因子VIII抑制物识别轻链，而本例中的抑制物与因子VIII的重链发生反应。
