慢性粒单核细胞白血病预后系统的循证评价

Evidence-based evaluation of prognostic systems in chronic myelomonocytic leukemia

慢性粒单核细胞白血病(chronic myelomonocytic leukemia,CMML)是一类具有骨髓增生异常综合征(myelodysplastic syndromes,MDS)和骨髓增殖性肿瘤(myeloid proliferative neoplasm,MPN)特征的恶性造血干细胞疾病,是最具侵袭性的慢性白血病之一。CMML患者的预后表现出高度异质性,现有的多种疾病分型、预后评分系统对患者的风险评估的能力仍不能令人满意。2016年版WHO指南推荐使用WHO或FAB分型系统区分患者亚型,欧洲白血病网(European LeukemiaNet,ELN)和欧洲血液学协会(European Hematology Association,EHA)建议优先使用GFM、Mayo Molecular、CPSS-mol模型对患者预后风险进行评估。选择恰当的分型、预后系统对CMML患者的风险进行评估仍是临床工作中面临的巨大挑战。

Chronic myelomonocytic leukemia(CMML)is one of the most aggressive clonal hematologic diseases,sharing features of both myelodysplastic syndromes(MDS)and chronic myeloid proliferative neoplasm(MPN).Prognosis of patients with CMML is highly heterogeneous,while risk-based stratification according to existing prognostic systems is still limited.According to the 2016 iteration of the World Health Organization(WHO)classification of myeloid neoplasms,both WHO and FAB are recommended for for the diagnosis and stratification of CMML.As for prognosis-based stratification scoring systems,the GFM,Mayo Molecular,CPSS-mol systems are primarily recommended by the European LeukemiaNet(ELN)and European Hematology Association(EHA).