Suetomo M, Suehisa E, Nishimura K, Toyokawa M, Toku M, Fushimi R, Amino N, Miyai K, Izumi M, Ohtsuka A
Central Laboratory for Clinical Investigation, Osaka University Hospital.
Rinsho Byori. 1991 Jul;39(7):748-52.
We examined an inhibitor to factor VIII in non-haemophilic patient who had been developed widely spread ecchymosis and intramuscular bleeding. He had no previous personal or family history of abnormal bleeding tendency. His laboratory data was all normal except examination for blood coagulation. Coagulation studies showed prolonged activated partial thromboplastin time (48 sec) and decreased factor VIII activity (8%). The activity of the inhibitor to factor VIII was demonstrated to be 4.0 Bethesda unit. By the studies of dilution and time response curve, this inhibitor was found to inhibit up to 90% of factor VIII activity but not 100%. This inhibitor was shown to be IgG by protein-A affinity chromatography. In addition, bleeding time was prolonged in the patient. The value of von Willebrand factor antigen was 200%, but that of Ristocetin cofactor was 93%. Since the gel filtration analysis indicated that this inhibitor also suppressed Ristocetin cofactor activity, the relatively low value of Ristocetin cofactor might occur through the action of the inhibitor. These data suggest that patient's inhibitor react to factor VIII high molecular subunit.
我们对一名非血友病患者进行了检查，该患者出现了广泛的瘀斑和肌肉内出血。他既往无个人或家族异常出血倾向史。除凝血检查外，他的实验室检查数据均正常。凝血研究显示活化部分凝血活酶时间延长（48秒），因子VIII活性降低（8%）。因子VIII抑制物的活性被证明为4.0贝塞斯达单位。通过稀释和时间反应曲线研究，发现该抑制物可抑制高达90%的因子VIII活性，但不能抑制100%。通过蛋白A亲和层析显示该抑制物为IgG。此外，该患者的出血时间延长。血管性血友病因子抗原值为200%，但瑞斯托霉素辅因子值为93%。由于凝胶过滤分析表明该抑制物也抑制瑞斯托霉素辅因子活性，瑞斯托霉素辅因子相对较低的值可能是通过该抑制物的作用而出现的。这些数据表明患者的抑制物与因子VIII高分子亚基发生反应。
