骨髓增生异常综合征（MDS）是一组异质的克隆性血液疾病，存在向急性髓系白血病（AML）转化的高风险。鉴定MDS中关键的遗传改变有助于我们对发病机制和演变的理解。近年来，发现MDS造血微环境中存在先天免疫和适应性免疫信号的活化，伴有异常的细胞因子分泌。同时明确了免疫调节失衡在MDS的发生和进展中起重要作用，尤其是破坏骨髓微环境，包括造血和基质组分。本综述的目的是探讨免疫细胞、免疫微环境和细胞因子在MDS发病机制中的作用。对这些变异机制的洞察可能有助于开发新的有效治疗手段以防止疾病进展。© 作者(们)，2023年。

Myelodysplastic syndrome (MDS) is a heterogeneous group of clonal hematological diseases and a high risk for transformation to acute myeloid leukemia (AML). The identification of key genetic alterations in MDS has enhanced our understanding of the pathogenesis and evolution. In recent years, it has been found that both innate and adaptive immune signaling are activated in the hematopoietic niche of MDS with aberrant cytokine secretion in the bone marrow microenvironment. It is also clear that immune dysregulation plays an important role in the occurrence and progression of MDS, especially the destruction of the bone marrow microenvironment, including hematopoiesis and stromal components. The purpose of this review is to explore the role of immune cells, the immune microenvironment, and cytokines in the pathogenesis of MDS. Insights into the mechanisms of these variants may facilitate the development of novel effective treatments to prevent disease progression.© The Author(s), 2023.