Shima M, Sawamoto Y, Kamisue M, Shibata Y, Tuzi S, Kuwabara M, Tanaka I, Tanizawa T, Tanaka A, Ueda M, Kakishita E, Yoshioka A
Department of Pediatrics, Nara Medical University, Japan.
Rinsho Ketsueki. 1996 Nov;37(11):1303-8.
A hemophilia A patient with high responder inhibitor had been treated by (activated) prothrombin complex concentrates (A) PCC and activated factor VII until the occurrence of intracranial bleeding at the age of 6 years. Since the inhibitor titer was decreased less than 1 Bethesda Units/ml, high dose of factor VIII was given followed by the infusions of factor VIII concentrates (100 units/kg) three times a week. In spite of previous episodes of anamnestic responses by factor VIII products before, the inhibitor titer did not increase and disappeared completely 6 months after the FVIII infusion therapy. The specific anti-factor VIII IgG subclasses of the inhibitor were IgG2 and IgG4. The inhibitor recognized both light and heavy chains. He have no bleeding episode for 6 months since the beginning of the prophylactic with factor VIII concentrates.
一名高反应性抑制物的甲型血友病患者曾接受（活化）凝血酶原复合物浓缩剂（A）PCC和活化凝血因子VII治疗，直至6岁时发生颅内出血。由于抑制物滴度下降至低于1贝塞斯达单位/毫升，给予高剂量的凝血因子VIII，随后每周三次输注凝血因子VIII浓缩剂（100单位/千克）。尽管之前使用凝血因子VIII产品时有过回忆反应发作，但在凝血因子VIII输注治疗6个月后，抑制物滴度并未升高且完全消失。该抑制物的特异性抗凝血因子VIII IgG亚类为IgG2和IgG4。该抑制物可识别轻链和重链。自开始使用凝血因子VIII浓缩剂进行预防治疗以来，他已有6个月未发生出血事件。
