Baugh R F, Hougie C
J Lab Clin Med. 1981 Jun;97(6):864-80.
A patient with a reduced response to the platelet-aggregating agent ristocetin and a prolonged bleeding time was found to have an abnormally high level of vWf as measured by Laurell immunoelectrophoresis. Characterization of the patient's vWf by disc-gel electrophoresis and crossed immunoelectrophoresis showed it to be indistinguishable from vWf isolated from normal plasma. The specific activity of the purified vWf in supporting RIPA was the same for both patient and normal vWf. The diminished RIPA was therefore not due to decreased levels of vWf or the presence of a modified form of the vWf. The patient's platelet-poor plasma inhibited the ristocetin-induced platelet aggregating activity of normal PRP, indicating the presence of an inhibitor. Fractionation of the plasma by ion-exchange chromatography showed the inhibitory activity to be in the patient's gamma globulin fraction. The gamma globulin was not directed against either the patient's vWf or against the patient's platelets but appeared to interfere with RIPA by binding ristocetin.
一名对血小板聚集剂瑞斯托霉素反应降低且出血时间延长的患者，经劳雷尔免疫电泳检测发现其血管性血友病因子（vWf）水平异常升高。通过圆盘凝胶电泳和交叉免疫电泳对该患者的vWf进行表征，结果显示其与从正常血浆中分离出的vWf无法区分。纯化后的vWf在支持瑞斯托霉素诱导的血小板聚集（RIPA）方面，患者和正常vWf的比活性相同。因此，RIPA降低并非由于vWf水平降低或存在修饰形式的vWf。该患者的乏血小板血浆抑制了正常富血小板血浆（PRP）的瑞斯托霉素诱导的血小板聚集活性，表明存在一种抑制剂。通过离子交换色谱对血浆进行分级分离显示，抑制活性存在于患者的γ球蛋白组分中。γ球蛋白并非针对患者自身的vWf或患者的血小板，而是似乎通过结合瑞斯托霉素来干扰RIPA。
