免疫失耐受在ITP自身抗体产生机制中的研究进展  

Mechanisms of loss of immune tolerance in the production of antiplatelet autoantibodies in primary immune thrombocytopenia

作　　者：翟菊萍[1] 何杨[2] 杨炳华[1] 

机构地区：[1]苏州大学附属第一医院,215006 [2]苏州大学附属第一医院,江苏省血液学研究所,215006

出　　处：《中华微生物学和免疫学杂志》2016年第10期790-794,共5页Chinese Journal of Microbiology and Immunology

基　　金：江苏省高校优势学科建设工程资助项目（YX21100213）

摘　　要：原发免疫性血小板减少症（primary immune thrombocytopenia，ITP）是一种器官特异性自身免疫出血性疾病。虽然ITP的病因至今还不明确，但一般认为对血小板自身抗原的免疫耐受丧失是ITP发病的根本原因。维持免疫耐受的机制主要包括自身反应性淋巴细胞的清除（凋亡）、受体编辑、克隆诱导无能和调节性免疫细胞的抑制作用等，调控机制一旦失效，将导致自身反应性淋巴的过度活化及增殖，最终导致靶向血小板和巨核细胞表面糖蛋白的自身抗体的产生。对ITP自身抗体产生机制的深入认识为其更好地诊断治疗提供理论依据。Primary immune ihrombocytopenia （ITP） is an organ-specific autoimmune hemorrhagic disease. The etiology of ITP is still unclear, but loss of immune tolerance to platelet surface antigens is con- sidered as a fundamental cause of ITP. Therapeutic strategies that prevent the activation and proliferation of autoreactive cells have been suggested, which includes clearance of autoreactive ceils （apoptosis） , receptor editing, induction of anergy and extrinsic cellular suppression. Failure at any of these steps may lead to the production of autoantibodies against platelet and megakaryocyte glycoproteins. An improved understanding of the mechanisms for autoantibody production will provide theoretical basis for optimal diagnosis and treatment of ITP.

关 键 词：原发免疫性血小板减少症 自身抗体 免疫耐受 

分 类 号：R558.2[医药卫生—血液循环系统疾病]