Jonas Brian A, Greenberg Peter L
Department of Internal Medicine, Division of Hematology and Oncology, University of California Davis School of Medicine, UC Davis Comprehensive Cancer Center, 4501 X Street, Suite 3016, Sacramento, CA 95817, United States.
Department of Internal Medicine, Division of Hematology, Stanford University School of Medicine, Stanford Comprehensive Cancer Center, 875 Blake Wilbur Drive, Stanford, CA 94305, United States.
Best Pract Res Clin Haematol. 2015 Mar;28(1):3-13. doi: 10.1016/j.beha.2014.11.001. Epub 2014 Nov 11.
The myelodysplastic syndromes (MDS) are a heterogeneous group of clonal myeloid haemopathies characterized by defective differentiation of haematopoietic cells and expansion of the abnormal clone. This leads to bone marrow failure with the resulting peripheral blood cytopenias and evolution to or toward acute myeloid leukaemia that characterize MDS clinically. The clinical heterogeneity of MDS has led several groups to analyze patient and clinical characteristics to develop prognostic scoring systems yielding estimates of overall and leukaemia-free survival to guide clinical decision-making. These models have evolved over time as our understanding of the pathogenesis, natural history, and treatment of MDS has improved. Rapid advances in flow cytometric analysis, adjuncts to standard metaphase cytogenetics, and gene mutation analysis are revolutionizing our understanding of MDS pathogenesis and prognosis. Despite the existence of multiple well-validated prognostic scoring systems, further refinements of current models with these new sources of prognostic data are needed and are described herein.
骨髓增生异常综合征（MDS）是一组异质性的克隆性髓系血液病，其特征为造血细胞分化缺陷及异常克隆扩增。这会导致骨髓衰竭，进而引起外周血细胞减少，并演变为急性髓系白血病或向其发展，这些在临床上构成了MDS的特征。MDS的临床异质性促使多个研究小组分析患者及临床特征，以开发预后评分系统，从而得出总生存期和无白血病生存期的估计值，为临床决策提供指导。随着我们对MDS发病机制、自然史和治疗的认识不断提高，这些模型也在不断演变。流式细胞术分析、标准中期细胞遗传学辅助技术以及基因突变分析的快速进展正在彻底改变我们对MDS发病机制和预后的认识。尽管存在多个经过充分验证的预后评分系统，但仍需要利用这些新的预后数据来源对当前模型进行进一步完善，本文对此进行了描述。
