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WHO 分类为基础的预后评分系统(WPSS)在骨髓增生异常综合征中的验证,并与修订后的国际预后评分系统(IPSS-R)进行比较。这是骨髓增生异常综合征预后国际工作组(IWG-PM)的一项研究。

Validation of WHO classification-based Prognostic Scoring System (WPSS) for myelodysplastic syndromes and comparison with the revised International Prognostic Scoring System (IPSS-R). A study of the International Working Group for Prognosis in Myelodysplasia (IWG-PM).

机构信息

1] Department of Hematology Oncology, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy [2] Department of Internal Medicine, University of Pavia, Pavia, Italy.

Hanusch Hospital, Boltzmann Institute for Leukemia Research, Vienna, Austria.

出版信息

Leukemia. 2015 Jul;29(7):1502-13. doi: 10.1038/leu.2015.55. Epub 2015 Feb 27.

Abstract

A risk-adapted treatment strategy is mandatory for myelodysplastic syndromes (MDS). We refined the World Health Organization (WHO)-classification-based Prognostic Scoring System (WPSS) by determining the impact of the newer clinical and cytogenetic features, and we compared its prognostic power to that of the revised International Prognostic Scoring System (IPSS-R). A population of 5326 untreated MDS was considered. We analyzed single WPSS parameters and confirmed that the WHO classification and severe anemia provide important prognostic information in MDS. A strong correlation was found between the WPSS including the new cytogenetic risk stratification and WPSS adopting original criteria. We then compared WPSS with the IPSS-R prognostic system. A highly significant correlation was found between the WPSS and IPSS-R risk classifications. Discrepancies did occur among lower-risk patients in whom the number of dysplastic hematopoietic lineages as assessed by morphology did not reflect the severity of peripheral blood cytopenias and/or increased marrow blast count. Moreover, severe anemia has higher prognostic weight in the WPSS versus IPSS-R model. Overall, both systems well represent the prognostic risk of MDS patients defined by WHO morphologic criteria. This study provides relevant in formation for the implementation of risk-adapted strategies in MDS.

摘要

对于骨髓增生异常综合征(MDS),风险适应的治疗策略是强制性的。我们通过确定新的临床和细胞遗传学特征的影响来改进基于世界卫生组织(WHO)分类的预后评分系统(WPSS),并将其预后能力与修订后的国际预后评分系统(IPSS-R)进行比较。考虑了一个未治疗的 MDS 患者群体 5326 例。我们分析了单个 WPSS 参数,并证实了 WHO 分类和严重贫血在 MDS 中提供了重要的预后信息。在包括新的细胞遗传学风险分层的 WPSS 和采用原始标准的 WPSS 之间发现了很强的相关性。然后,我们将 WPSS 与 IPSS-R 预后系统进行了比较。WPSS 和 IPSS-R 风险分类之间存在高度显著的相关性。在形态学评估的造血谱系发育不良数量未反映外周血细胞减少症和/或骨髓原始细胞计数增加的严重程度的较低风险患者中,确实存在差异。此外,严重贫血在 WPSS 中比在 IPSS-R 模型中具有更高的预后权重。总体而言,这两个系统都很好地代表了 WHO 形态学标准定义的 MDS 患者的预后风险。本研究为 MDS 中实施风险适应策略提供了相关信息。

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