原发免疫性血小板减少症发病机制的研究进展

Research Progress in Pathogenesis of Primary Immune Thrombocytopenia

原发免疫性血小板减少症(ITP)的临床症状多样,发病率逐年升高,其发病机制复杂,与免疫介导的功能障碍密切相关,主要由于血小板自身抗原免疫耐受性降低,导致体液和细胞免疫过度活化,介导血小板清除加速及生成不足。近年发现,去唾液酸化、固有免疫应答失常、肠道菌群失调、微RNA差异化表达和程序性细胞死亡受体1、髓系来源抑制细胞、缺氧诱导因子-1α等新生物细胞分子的异常表达及相关作用途径同样参与影响ITP的发生发展。未来,多靶点联合治疗及个体化治疗将成为ITP治疗的趋势及研究重点。

The clinical symptoms of primary immune thrombocytopenia(ITP)are diverse,and its incidence is increasing year by year.Its pathogenesis is complex,and is closely related to immune-mediated dysfunction,mainly due to the decrease in the immune tolerance of platelet autoantigens,resulting in hyperactivation of humoral and cellular immune,mediating accelerated platelet clearance and insufficient production.In recent years,it′s discovered that desialylation,abnormal innate immune response,intestinal flora imbalance,differential expression of microRNA,abnormal expression of newly discovered biological cells and moleculars such as programmed cell death protein 1,myeloid-derived suppressor cells,and hypoxia-inducible factor-1α,and related pathways are also involved in the occurrence and development of ITP.In the future,multi-target combined therapy and individualized therapy will become the trend and research focus of ITP treatment.