骨髓增生异常综合征（myelodysplastic syndrome，MDS）是血液系统中较为常见的一组髓系恶性克隆性疾病。不同分型的MDS患者，其临床表现及自然病程各异，加上历史上不同分型系统及版本的迭代，对其诊断、分型及治疗方案选择造成了一定困扰。随着分子生物学技术的进步，来那度胺、罗特西普、维奈克拉等药物的使用，近年来MDS分型标准与预后积分系统在不断完善细化。在2016年WHO分型标准中，首次引入了分子相关的分型标准（SF3B1），并对5q-进行了再定义，强调了驱动突变的概念。2022年WHO分型标准则在原有基础上更加突出了分子生物学特征，同时关于SF3B1、5q-的亚型也被保留在2022年的WHO分型标准中，并新增了新的分子相关分型亚型（bi-TP53）。新提出的MDS分子国际预后评分系统（the Molecular International Prognostic Scoring System，IPSS-M）则在2012年提出的MDS修订版国际预后评分系统（the Revised International Prognostic Scoring System，IPSS-R）的基础上，引入了分子突变作为新的积分标准，并结合了既有的形态学和细胞遗传学特征，使得MDS患者的危险因素分层更加精准和个体化。随着分子靶向药物的不断推出和检验技术的提升，IPSS-M必将不断更新，笔者对近些年的MDS分型标准以及预后分层系统的进展和临床意义进行总结。

Myelodysplastic syndrome (MDS) is a relatively common group of myeloid malignant clonal diseases in the blood system. Different types of MDS patients have different clinical manifestations and natural course, as well as the iterations of different classification systems and versions in history, resulting distress in diagnosis, classification and treatment options. With the advancement of molecular biology technology and the use of Lenalidomide, Luspatercept, Venetoclax and other drugs, the classification criteria and prognostic scoring system of myelodysplastic syndrome have been continuously refined in recent years. In the 2016 WHO classification criteria, a molecular-related classification criterion (SF3B1) was introduced for the first time, and 5q- was redefined, emphasizing the concept of driver mutation. The 2022 WHO classification standard has more prominent molecular biological characteristics based on the original, and the classification of SF3B1 and 5q- has also been retained in the 2022 WHO classification standard. A new molecular correlation classification standard (bi-TP53) has been added to the 2022 WHO classification standard to further improve the classification of MDS patients by clinicians and researchers, reflecting the new trend of future genomics research that guide the precision medicine treatment of MDS. At the same time, the prognostic scoring system for MDS patients is constantly being updated. Based on the Revised International Prognostic Scoring System (IPSS-R) for Myelodysplastic Syndromes proposed in 2012, the newly proposed Molecular International Prognostic Scoring System (IPSS-M) for myelodysplastic Syndrome introduces a new prognostic scoring system for MDS patients, the stratification of risk factors in MDS patients is more precise and individualized. With the improvement of molecular targeted drugs and testing technology, IPSS-M will continue to be updated. To this end, we will summarize the classification criteria of MDS in recent years, as well as the progress and clinical significance of the prognostic stratification system.