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出现因子Ⅷ抑制物是血友病患者治疗时的一种严重且往往威胁生命的并发症。在大多数病例,这种循环抗体的存在使出血患者对常规的因子Ⅷ替代治疗失效。据推测,抑制物的产生是由于对外源性因子Ⅷ的免疫反应的个体差异所致,而免疫原本身引起的作用是次要的。因子Ⅷ抑制物为异质性的循环免疫球蛋白,常为IgG_1和IgG_4,对因子Ⅷ复合物的促凝成份(Ⅷ:C)有特异性。通过抑制物定量分析,发现这些抗体可与Ⅷ:C起中和反应,且与作用时间有关。某些患者通过回忆免疫反应可导致抗体的效价明显增高,但另一些
The presence of factor VIII inhibitors is a serious and often life-threatening complication in the treatment of hemophiliacs. In the majority of cases, the presence of such circulating antibodies renders bleeding patients ineffective with conventional Factor VIII replacement therapy. It is speculated that the production of inhibitors is due to individual differences in the immune response to exogenous factor VIII, whereas the effects caused by the immunogen itself are secondary. Factor VIII inhibitors are heterogeneous circulating immunoglobulins, often IgG1 and IgG4, that are specific for the procoagulant component of Factor VIII complex (Ⅷ: C). By quantitation of inhibitors, these antibodies were found to neutralize with Ⅷ: C, depending on the duration of action. Some patients recall the immune response can lead to significantly higher antibody titer, but others