Risk factors and their relationship to prognosis in myelodysplastic syndromes

Risk factors and their relationship to prognosis in myelodysplastic syndromes
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DOI:
10.1016/s0145-2126(98)00040-x
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发表时间:
1998-05-01
期刊:
影响因子:
2.7
通讯作者:
Greenberg, PL
Greenberg, PL
中科院分区:
医学3区
文献类型:
--
作者:
Greenberg, PL

文献摘要

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最近的努力已经针对改善的方法预测骨髓增生异常综合征(MDS)患者的临床结果。本文综述了国际MDS风险分析研讨会产生的共识,预后,基于风险的分析系统的发展。在研讨会上,细胞遗传学,形态学和临床数据相结合,并从一个相对较大的组与原发性MDS患者进行整理。使用数据集评价关键预后变量。基于这些发现,与其他系统相比,开发了国际预后评分系统(IPSS),并显示出对MDS患者的生存和急性髓性白血病演变提供更准确的评估。这种改善是由于工作坊模型的几个特点:更精确的细胞遗传学分类,包括血细胞减少,改善骨髓原始细胞百分比的细分,四个亚组定义的结果,以及单独的年龄分层。IPSS应导致更好地定义MDS的临床结局,并为未来的研究提供一个框架,以确定分子决定因素(如癌基因、肿瘤抑制基因、细胞因子表达和反应性)在评估MDS中的可能作用。IPSS可能会被证明是有用的设计和分析的治疗试验在MDS以及患者管理。(C)1998爱思唯尔科技有限公司。保留所有权利。
Recent efforts have been directed at improving the methodology for predicting clinical outcomes in patients with myelodysplastic syndromes (MDS). This review focuses on the development of a consensual, prognostic, risk-based analysis system generated by the International MDS Risk Analysis Workshop. In the workshop, cytogenetic, morphological, and clinical data were combined and collated from a relatively large group of patients with primary MDS. Critical prognostic variables were evaluated using the data set. Based on these findings, the International Prognostic Scoring System (IPSS) was developed, compared with other systems, and shown to provide more accurate prognoses regarding survival and evolution to acute myeloid leukemia in MDS patients. The improvement was due to several features of the workshop model: more refined cytogenetic categorization, inclusion of cytopenias, improved subdivision of marrow blast percentages, four subgroups defining outcome, and separate stratification for age. The IPSS should result in better-defined clinical outcomes in MDS and provide a framework for future studies determining the possible role of molecular determinants (e.g. oncogenes, tumor suppressor genes, cytokine expression and responsiveness) for evaluating prognoses. The IPSS will likely prove useful in the design and analysis of therapeutic trials in MDS as well as in patient management. (C) 1998 Elsevier Science Ltd. All rights reserved.