Hemophilia factor VIII therapy. B- and T-cell tolerance: from basic concepts to clinical practice.
Hemophilia factor VIII therapy. B- and T-cell tolerance: from basic concepts to clinical practice.
复制标题
血友病因子 VIII 治疗。
作者：
J. Saint
对 B 细胞和 T 细胞耐受性发展机制的理解的最新进展导致了对可能适用于临床环境（例如具有抑制剂的血友病患者）的可能方法的重新评估。因此，现在很清楚，许多机制以这样的方式运作，以主动诱导B细胞或T细胞无反应性，甚至特异性地删除外周细胞。 B 细胞和 T 细胞库以及外周细胞池固有的特征在耐受诱导的效率和维持中发挥着重要作用。本手稿的目的是简要回顾耐受诱导机制，以便为理解当前和未来在血友病 A 患者中实现因子 VIII 无反应的尝试提供一个框架。
Recent advances in the understanding of the mechanisms by which tolerance develops in both B- and T- cells have led to a re-appraisal of possible methods which could be applicable in clinical settings such as hemophilia patients with inhibitors. Thus, it is now clear that a number of mechanisms operate in such a way as to actively induce anergy of B- or T- cells, or even to specifically delete cells in the periphery. The characteristics inherent to the B- and T- cell repertoires and peripheral pools play a major role in the efficiency and maintenance of tolerance induction. The purpose of this manuscript is to review the mechanisms of tolerance induction briefly so as to provide a framework for the understanding of current and future attempts to achieve Factor VIII unresponsiveness in hemophilia A patients.