In 2022, two novel classification systems for myelodysplastic syndromes/neoplasms (MDS) have been proposed: the International Consensus Classification (ICC) and the 2022 World Health Organization (WHO-2022) classification. These two contemporary systems exhibit numerous shared features but also diverge significantly in terminology and the definition of new entities. Thus, we retrospectively validated the ICC and WHO-2022 classification and found that both systems promoted efficient segregation of this heterogeneous disease. After examining the distinction between the two systems, we showed that a peripheral blood blast percentage ≥ 5% indicates adverse survival. Identifying MDS/acute myeloid leukemia with MDS-related gene mutations or cytogenetic abnormalities helps differentiate survival outcomes. In MDS, not otherwise specified patients, those diagnosed with hypoplastic MDS and single lineage dysplasia displayed a trend of superior survival compared to other low-risk MDS patients. Furthermore, the impact of bone marrow fibrosis on survival was less pronounced within the ICC framework. Allogeneic transplantation appears to improve outcomes for patients diagnosed with MDS with excess blasts in the ICC. Therefore, we proposed an integrated system that may lead to the accurate diagnosis and advancement of future research for MDS. Prospective studies are warranted to validate this refined classification.

2022 年，提出了两种新的骨髓增生异常综合征/肿瘤 （MDS） 分类系统：国际共识分类 （ICC） 和 2022 年世界卫生组织 （WHO-2022） 分类。这两个当代系统表现出许多共同特征，但在术语和新实体的定义上也存在显着差异。因此，我们回顾性验证了 ICC 和 WHO-2022 分类，发现这两个系统都促进了这种异质性疾病的有效分离。在检查了两个系统之间的区别后，我们发现外周血原始细胞百分比≥ 5% 表明生存率不佳。识别具有 MDS 相关基因突变或细胞遗传学异常的 MDS/急性髓系白血病有助于区分生存结局。在 MDS 中，未另行说明的患者，与其他低风险 MDS 患者相比，那些被诊断为发育不良 MDS 和单系异型增生的患者表现出更高的生存率趋势。此外，在 ICC 框架内，骨髓纤维化对生存率的影响不太明显。同种异体移植似乎可以改善 ICC 中原始细胞过多的 MDS 患者的预后。因此，我们提出了一个综合系统，可能会导致 MDS 的准确诊断和未来研究的进步。需要前瞻性研究来验证这种精细的分类。