Why do inhibitors develop? Principles of and factors influencing the risk for inhibitor development in haemophilia
Why do inhibitors develop? Principles of and factors influencing the risk for inhibitor development in haemophilia
复制标题
DOI：
10.1111/j.1365-2516.2006.01261.x
复制
发表时间：
2006-07-01
期刊：
影响因子：
3.9
通讯作者：
Astermark, J
中科院分区：
文献类型：
--
作者：
Astermark, J
在感染后时代，抑制性同种抗体的形成是血友病患者替代治疗中最严重和最昂贵的并发症。随着对该领域知识的增加，免疫反应的复杂性变得越来越明显。抗体的产生是抗原呈递细胞、T淋巴细胞和b淋巴细胞之间复杂的多因子相互作用的结果。细胞表面分子的遗传易感性，如主要组织相容性复合体、t细胞受体和细胞因子受体，以及各种免疫调节分子对结果有重要影响。此外，环境因素可能影响抑制剂发展的风险。综述了目前抑制剂开发的概念。更好地了解所涉及的病理生理机制将促进未来治疗的改进，并有希望提供一个机会来预防这种并发症的治疗。
The formation of inhibitory alloantibodies is, in the postinfectious era, the most severe and costly complication of replacement therapy in patients with haemophilia. The complexity of the immune response to the infused factor becomes more and more obvious as knowledge in the area increases. Antibodies develop as a result of a complex multi-factorial interaction between antigen-presenting cells, T- and B-lymphocytes. Genetic susceptibility of cell surface molecules, such as the major histocompatibility complex, the T-cell receptor and cytokine receptors, as well as various immunomodulatory molecules have a major impact on the outcome. In addition, environmental factors probably influence the risk of inhibitor development. The current concept of inhibitor development is reviewed. A better understanding of the pathophysiological mechanisms involved will facilitate improvement of therapy in the future, and hopefully provide an opportunity to prevent this complication of treatment.