Ballard J O, Sanders J C, Eyster M E
Thromb Haemost. 1981 Feb 23;45(1):68-72.
Inhibitors to factor VIII (anti-F VIII) developing in patients with classic haemophilia have apparent specificity for the factor VIII procoagulant activity (VIII : C), rather than the factor VIII-related antigen (VIII : AGN) and von Willebrand factor (VIII : vWf) regions of the factor VIII complex. Since procoagulant function is absent following in vitro clotting, but serum retains VIII : AGN/vWf properties, we searched for differences in VIII : AGN and VIII : vWf of inhibitor serum that might relate to the presence of anti-F VIII. Rocket immunoelectrophoresis and the washed platelet ristocetin assay were performed on the plasma and serum of nine haemophiliacs with inhibitors, 23 non-inhibitor haemophiliacs and six normal subjects. Unlike normal and non-inhibitor haemophilic sera, that from five of nine inhibitor patients demonstrated absent VIII : vWf and significantly lower VIII : AGN (p less than 0.05). Furthermore, VIII : AGN of faster mobility was detected on crossed immunoelectrophoresis of the sera of three inhibitor patients. Thrombin clotting of plasma from haemophiliacs with high titer anti-F VIII was associated with a greater loss of VIII : vWf than seen with non-inhibitor haemophilic plasma. This effect was independent of the presence of platelets. These data indicate that in vitro clotting is associated with alteration in the serum VIII : AGN/vWf of some haemophiliacs with anti-F VIII.
在典型血友病患者体内产生的凝血因子VIII抑制剂（抗F VIII）对凝血因子VIII的促凝活性（VIII : C）具有明显的特异性，而非针对凝血因子VIII复合物中的凝血因子VIII相关抗原（VIII : AGN）和血管性血友病因子（VIII : vWf）区域。由于体外凝血后促凝功能缺失，但血清仍保留VIII : AGN/vWf特性，我们研究了抑制剂血清中VIII : AGN和VIII : vWf的差异，这些差异可能与抗F VIII的存在有关。对9例有抑制剂的血友病患者、23例无抑制剂的血友病患者以及6名正常受试者的血浆和血清进行了火箭免疫电泳和洗涤血小板瑞斯托霉素试验。与正常和无抑制剂的血友病血清不同，9例有抑制剂患者中的5例血清显示VIII : vWf缺失且VIII : AGN显著降低（p < 0.05）。此外，在3例有抑制剂患者的血清交叉免疫电泳中检测到迁移速度更快的VIII : AGN。高滴度抗F VIII的血友病患者血浆经凝血酶凝血后，与无抑制剂的血友病血浆相比，VIII : vWf的损失更大。这种效应与血小板的存在无关。这些数据表明，体外凝血与一些有抗F VIII的血友病患者血清VIII : AGN/vWf的改变有关。
