Allain J P, Gaillandre A, Frommel D
Thromb Haemost. 1981 Jun 30;45(3):285-9.
Factor VIII complex and its interaction with antibodies to factor VIII have been studied in 17 non-haemophilic patients with factor VIII inhibitor. Low VIII:C and high VIIIR:Ag levels were found in all patients. VIII:WF levels were 50% of those of VIIIR:Ag, possibly related to an increase of poorly aggregated and electrophoretically fast moving VIIIR:Ag oligomers. Antibody function has been characterized by kinetics of VIII:C inactivation, saturability by normal plasma and the slope of the affinity curve. Two major patterns were observed: 1) Antibodies from 6 patients behaved similarly to those from haemophiliacs by showing second order inhibition kinetics, easy saturability and steep affinity slope (greater than 1). 2) Antibodies from other patients, usually with lower titres, inactivated VIII:C according to complex order kinetics, were not saturable, and had a less steep affinity slope (less than 0.7). In native plasma, or after mixing with factor VIII concentrate, antibodies of the second group did not form immune complexes with the whole factor VIII molecular complex. However, dissociation procedures did release some antibodies from apparently low molecular weight complexes formed in vivo or in vitro. For appropriate management of non-haemophilic patients with factor VIII inhibitor, it is important to determine the functional properties of their antibodies to factor VIII.
对17例患有VIII因子抑制剂的非血友病患者的VIII因子复合物及其与VIII因子抗体的相互作用进行了研究。所有患者均发现VIII:C水平低而VIIIR:Ag水平高。VIII:WF水平为VIIIR:Ag的50%，这可能与聚集性差且电泳迁移快的VIIIR:Ag寡聚体增加有关。通过VIII:C失活动力学、正常血浆的饱和性以及亲和力曲线的斜率对抗体功能进行了表征。观察到两种主要模式：1) 6例患者的抗体表现与血友病患者的抗体相似，表现为二级抑制动力学、易于饱和且亲和力斜率陡峭（大于1）。2) 其他患者的抗体通常滴度较低，根据复合级动力学使VIII:C失活，不可饱和，且亲和力斜率较平缓（小于0.7）。在天然血浆中，或与VIII因子浓缩物混合后，第二组抗体未与整个VIII因子分子复合物形成免疫复合物。然而，解离程序确实从体内或体外形成的明显低分子量复合物中释放出了一些抗体。对于患有VIII因子抑制剂的非血友病患者进行适当管理，确定其VIII因子抗体的功能特性很重要。
