MDS prognostic scoring systems – past, present, and future.
MDS prognostic scoring systems – past, present, and future.
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DOI：
10.1016/j.beha.2014.11.001
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发表时间：
2015-03
影响因子：
2.1
通讯作者：
Greenberg, Peter L.
中科院分区：
文献类型：
--
作者：
Jonas, Brian A.;Greenberg, Peter L.
骨髓增生异常综合征(MDS)是一组以造血细胞分化缺陷和异常克隆扩张为特征的异质性克隆性髓系血液病。这会导致骨髓衰竭，导致外周血细胞减少，并演变为或进展为急性髓系白血病，这是MDS的临床特征。MDS的临床异质性导致了几个小组分析患者和临床特征，以开发预后评分系统，得出总体和无白血病生存率的估计，以指导临床决策。随着我们对MDS的发病机制、自然病程和治疗的了解有所提高，这些模型也随着时间的推移而演变。流式细胞术分析、标准中期细胞遗传学的辅助手段和基因突变分析的快速发展正在彻底改变我们对MDS发病机制和预后的理解。尽管存在多个经过良好验证的预后评分系统，但仍描述了使用这些新的预后数据来源对当前模型进行进一步改进所需的改进。
The myelodysplastic syndromes (MDS) are a heterogeneous group of clonal myeloid haemopathies characterized by defective differentiation of hematopoietic cells and expansion of the abnormal clone. This leads to the bone marrow failure with resulting peripheral blood cytopenias and evolution to or toward acute myeloid leukaemia that characterise MDS clinically. The clinical heterogeneity of MDS has led several groups to analyse 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 needed refinements of current models with these new sources of prognostic data are described.
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期刊：
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