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免疫异常在骨髓增生异常综合征(MDS)疾病的发生、发展中发挥重要作用。目前，对于MDS免疫异常机制的探讨主要集中于自身免疫反应与自身炎症反应。自身免疫反应指自身反应性T细胞及高滴度的自身抗体参与的异常免疫状态，而自身炎症反应则主要与髓系来源免疫细胞的固有免疫反应密切相关。既往关于MDS免疫异常机制的研究主要集中于自身免疫反应，尤其是T细胞介导的造血干细胞抑制方面。而近年的研究发现，固有免疫信号通路活化、骨髓源性抑制细胞(MDSC)、细胞焦亡等固有免疫异常等自身炎症反应，亦在MDS的病理生理学机制中发挥重要作用，这为MDS的治疗提供了新的思路和药物研发方向。笔者拟就MDS患者自身免疫反应与自身炎症反应免疫异常机制的研究最新进展进行介绍。“,”Abnormal immunity plays an important role in etiology and evolution of myelodysplastic syndromes (MDS). The studies on abnormal immunity of MDS are mainly focused on autoimmune response and autoimmune inflammation reaction. Autoimmune response refers to the abnormal immune state with excess auto-reactive T cells and high titer autoantibodies, while autoimmune inflammation reaction is mainly related to innate immune response involving myeloid-derived cells. Previous researches about immunological abnormalities of MDS often focused on autoimmune response, especially T cell mediated inhibition of bone marrow stem cell proliferation. Recently more studies have found that autoimmune inflammation reaction, including activation of innate immune signaling pathways, myeloid derived suppressor cell (MDSC) and pyroptosis, which play important roles in pathophysiology of MDS and provide a new thought and drug research direction for treatment of MDS. This article summarizes the advances in researches on mechanisms of autoimmune response and autoimmune inflammation reaction in patients with MDS.