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Immune Dysregulation in Immune Thrombocytopenia (ITP)

机译:免疫失调在免疫性血小板减少症(ITp)

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摘要

Immune thrombocytopenia (ITP) is a bleeding disorder characterized by low platelet counts due to decreased platelet production as well as increased platelet destruction by autoimmune mechanisms. A shift toward Th1 and possibly Th17 cells together with impaired regulatory compartment, including Tregs and Bregs, have been reported, suggesting a generalized immune dysregulation in ITP. Interestingly, several treatments including the use of thrombopoietic agents appear to be associated with improvement in the regulatory compartment. Understanding how Th1/Th17/Treg differentiation and expansion are controlled is central to uncovering how autoimmunity may be sustained in chronic ITP and reversed following response to therapy. In this review, we will summarize the recent findings on the state of the Breg and Treg compartments in ITP, the role of monocyte subsets in the control of Th/Treg expansion and our working model of how the regulatory compartment may impact response to treatment and the means by which this information may guide therapy in ITP patients in the future.
机译:免疫性血小板减少症(ITP)是一种出血性疾病,其特征是由于血小板生成减少以及自身免疫机制导致的血小板破坏增加,血小板计数低。已经报道了向Th1细胞和可能的Th17细胞的转移以及包括Tregs和Bregs在内的调节区受损,表明ITP中普遍存在免疫失调。有趣的是,包括使用血小板生成剂在内的几种治疗方法似乎与调节室的改善有关。了解如何控制Th1 / Th17 / Treg的分化和扩增是揭示慢性ITP中如何维持自身免疫并在治疗反应后逆转自身免疫的关键。在本综述中,我们将总结有关ITP中Breg和Treg隔室状态,单核细胞亚群在Th / Treg扩展控制中的作用以及我们关于调节隔室如何影响治疗反应的工作模型的最新发现。这种信息将来可用于指导ITP患者治疗的手段。

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