Toronto Platelet Immunobiology Group, University of Toronto, Toronto, ON, Canada; Keenan Research Centre in the Li Ka Shing Knowledge Institute of St. Michael's Hospital, Toronto, ON, Canada; Canadian Blood Services, University of Toronto, Toronto, ON, Canada.
Br J Haematol. 2013 Oct;163(1):10-23. doi: 10.1111/bjh.12480. Epub 2013 Aug 12.
Over the past decades, a wealth of information has been reported about the pathogenic features of immune thrombocytopenia (ITP). To this day, however, it is unclear whether the immune abnormalities associated with ITP play causative roles in the disease or are secondary epiphenomena brought on by the inflammatory processes that are associated with the disorder. Like the majority of all autoimmune diseases, ITP is an organ-specific disease and abnormalities in immune cell types, such as antigen-presenting cells (APC), T cells and B cells have been shown to play some sort of role in the initiation and/or perpetuation of the disease. This review will discuss recent advances in understanding three immune cells important in ITP pathophysiology: APC, T cells and B cells, and will review how they interact with each other to initiate and perpetuate ITP, particularly the chronic form of the disorder. It will also focus on new data related to the genetics of the disorder and discuss relevant animal models of ITP.
在过去的几十年中,已经有大量关于免疫性血小板减少症 (ITP) 的发病特征的信息被报道。然而,到目前为止,仍然不清楚与 ITP 相关的免疫异常是否在疾病中起因果作用,或者它们是否是与该疾病相关的炎症过程引起的继发现象。与大多数自身免疫性疾病一样,ITP 是一种器官特异性疾病,免疫细胞类型的异常,如抗原提呈细胞 (APC)、T 细胞和 B 细胞,已被证明在疾病的起始和/或持续中发挥某种作用。这篇综述将讨论在理解 ITP 病理生理学中三种重要的免疫细胞:APC、T 细胞和 B 细胞方面的最新进展,并将综述它们如何相互作用以引发和持续 ITP,特别是该疾病的慢性形式。它还将重点关注与该疾病的遗传学相关的新数据,并讨论 ITP 的相关动物模型。