一名患有典型血友病和C2缺乏症患者对凝血因子VIII的脱敏反应。

Desensitization to factor VIII in a patient with classic hemophilia and C2 deficiency.

作者信息

Jamieson D M, Stafford C T, Maloney M J, Lutcher C L

出版信息

Ann Allergy. 1987 Mar;58(3):215-20.

Abstract

Factor VIII therapy has been reported to cause anaphylactic reactions in patients with hemophilia. Desensitization attempts have been complicated by severe allergic reactions that have prevented the achievement of protective factor VIII levels. We report successful administration of factor VIII by a graded dose desensitization protocol in a 36-year-old man with hemophilia A who had previously experienced anaphylactic reactions to factor VIII infusions. The reactions were manifested by urticaria, choking, and bronchospasm and were not prevented by pretreatment with antihistamines and corticosteroids. Intradermal skin test with factor VIII was positive. Serum levels of circulating immune complexes were slightly elevated. Persistently low serum C2 levels were consistent with genetic C2 deficiency. These findings suggest the possibility of Type I (IgE mediated) and Type III (immune complex) immunopathogenic mechanisms. Our experience suggests that administration of factor VIII by graded dose desensitization protocol may offer a practical therapeutic approach for management of hemorrhage in patients with classic hemophilia who are allergic to factor VIII.

摘要

据报道，凝血因子 VIII 疗法可导致血友病患者发生过敏反应。脱敏治疗尝试因严重过敏反应而变得复杂，这些反应阻碍了达到保护性凝血因子 VIII 水平。我们报告了一名 36 岁的甲型血友病男性患者，此前对凝血因子 VIII 输注有过敏反应，通过分级剂量脱敏方案成功给予了凝血因子 VIII。反应表现为荨麻疹、窒息和支气管痉挛，并且使用抗组胺药和皮质类固醇进行预处理也无法预防。凝血因子 VIII 的皮内皮肤试验呈阳性。循环免疫复合物的血清水平略有升高。血清 C2 水平持续偏低与遗传性 C2 缺乏一致。这些发现提示了 I 型（IgE 介导）和 III 型（免疫复合物）免疫致病机制的可能性。我们的经验表明，通过分级剂量脱敏方案给予凝血因子 VIII 可能为对凝血因子 VIII 过敏的典型血友病患者的出血管理提供一种实用的治疗方法。