Inhibitors: resolving diagnostic and therapeutic dilemmas
Inhibitors: resolving diagnostic and therapeutic dilemmas
复制标题
DOI：
10.1046/j.1365-2516.2002.00626.x
复制
发表时间：
2002-05-01
期刊：
影响因子：
3.9
通讯作者：
Dimichele, D
中科院分区：
文献类型：
--
作者：
Dimichele, D
血友病A和B患者目前预期的良好生活质量可能会因抑制剂（一种针对因子VIII或IX的多克隆高亲和力IgG抗体）的不可预测的发展而急剧改变。本文讨论了我们目前对抑制剂的认识：（1）问题的程度和性质，包括其发病率和患病率，诊断选择，以及个体免疫反应的特点;（2）可能的潜在病因学因素，包括遗传和环境因素;和（3）现有和未来的治疗选择，用于出血的中期治疗和正常凝血因子恢复和存活的最终恢复。目前和未来的免疫耐受策略也进行了审查。
The good quality of life now anticipated by individuals with haemophilia A and B can be drastically altered by the unpredictable development of an inhibitor, a polyclonal high-affinity IgG antibody directed against factor VIII or IX. This paper discusses our current state of knowledge about inhibitors within the context of: (1) the extent and nature of the problem, including its incidence and prevalence, diagnostic options, and the characteristics of the individual immunologic response; (2) possible underlying aetiological factors, both inherited and situational; and (3) existing and future therapeutic options for both the interim treatment of haemorrhage and the ultimate restoration of normal clotting factor recovery and survival. Current and future strategies for immune tolerisation are also reviewed.