VIII因子抑制剂的管理

Management of factor VIII inhibitors.

作者信息

Acharya Suchitra S, DiMichele Donna M

机构信息

Department of Pediatrics, New York Weill Center, Weill Medical College of Cornell University, 525 East 68th Street, P695, New York, NY 10021, USA.

出版信息

Best Pract Res Clin Haematol. 2006;19(1):51-66. doi: 10.1016/j.beha.2005.03.002.

DOI:10.1016/j.beha.2005.03.002

PMID:16377541

Abstract

The development of inhibitory alloantibodies to factor VIII is arguably one of the most severe and important complications of clotting factor concentrate exposure in haemophilia A. The development of an inhibitor compromises the ability to effectively manage haemorrhage, resulting in a greater rate of disability, complications and costs of therapy. This chapter briefly reviews the epidemiology, immunobiology, and laboratory evaluation of inhibitors. It discusses the therapeutic approach and management of inhibitors in various clinical settings and also focuses on inhibitor eradication practices (immune tolerance) and newer experimental strategies with potential clinical application for inhibitor prevention.

摘要

产生针对凝血因子 VIII 的抑制性同种抗体可以说是甲型血友病患者接触凝血因子浓缩物最严重且最重要的并发症之一。抑制性抗体的产生会损害有效控制出血的能力，导致更高的致残率、并发症发生率及治疗成本。本章简要回顾了抑制性抗体的流行病学、免疫生物学及实验室评估。讨论了在各种临床情况下针对抑制性抗体的治疗方法和管理，还重点介绍了抑制性抗体根除措施（免疫耐受）以及具有预防抑制性抗体潜在临床应用价值的新实验策略。