免疫性血小板减少性紫癜的发病机制

免疫性血小板减少性紫癜(ITP)是一组以血小板减少为特征的自身免疫紊乱性疾病群.既往认为,ITP发病机制为经典体液免疫机制,即通过自身抗体作用于未成熟血小板,使其在单核巨噬系统被吞噬所致,并可影响巨核细胞和血小板的发育成熟.目前,细胞免疫在ITP发病机制中的作用越来越受到重视,血小板、巨核细胞的细胞凋亡异常对ITP发病有影响,而一些细胞亚群比例异常和细胞因子水平异常被证实存在于ITP患者中,有关ITP患者基因的相关研究也取得了一定进展.除此之外,神经轴突导向分子(Sema)5A与维生素D3的异常,也参与ITP的发病.笔者拟主要从体液免疫、细胞免疫、细胞凋亡、基因表达、氧化应激等方面,对ITP发病机制的最新研究进展进行阐述.

Pathogenesis of immune thrombocytopenic purpura

Immune thrombocytopenic purpura(ITP) is a common autoimmune disorder characterized by isolated thrombocytopenia.Previous studies showed that autoantibodies bind to platelets and megakaryocytes playing a significant role in phagocytosis and depressing their maturation,which was the classic humoral immune mechanism.At present,the role of cellular immunity in the pathogenesis of ITP is more and more important.The abnormality of apoptosis of platelets and megakaryocytes also affects the incidence of ITP,abnormal protein of some kinds of cell subsets and abnormal levels of cytokines are confirmed in patients with ITP,and related researches on the gene of ITP patients has also made some progress.In addition,semaphorin (Sema) 5A and vitamin D3 abnormalities are also involved in pathogenesis of ITP.This article summarizes the research progress of ITP pathogenesis mainly from the humoral immunity,cellular immunity,apoptosis,gene expression,oxidative stress and other aspects.