Prognostic scoring systems for myelodysplastic syndromes (MDS) in a population-based setting: a report from the Swedish MDS register

Prognostic scoring systems for myelodysplastic syndromes (MDS) in a population-based setting: a report from the Swedish MDS register
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DOI:
10.1111/bjh.15243
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发表时间:
2018-06-01
影响因子:
6.5
通讯作者:
Ejerblad, Elisabeth
Ejerblad, Elisabeth
中科院分区:
医学2区
文献类型:
--
作者:
Berggren, Daniel Moreno;Folkvaljon, Yasin;Ejerblad, Elisabeth

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骨髓增生异常综合征(MDS)具有高度可变的结局,预后评分系统是风险评估和指导治疗决策的重要工具。然而,很少有基于人群的研究比较不同评分系统的价值。使用瑞典全国人口MDS登记的数据,我们验证了国际预后评分系统(IPSS)、修订的IPSS(IPSS-R)和世界卫生组织(WHO)基于分类的预后评分系统(WPSS)。我们还提供了基于人群的发病率、临床特征,包括详细的细胞遗传学和登记结果的数据。这项研究涵盖了2009至2013年间登记的1329名患者,其中14%患有与治疗相关的MDS(t-MDS)。根据MDS登记,瑞典每年的MDS粗发病率为每100000名居民中有2.9%。IPSS-R的预后能力明显好于IPSS(P<0001)。IPSS-R的预后能力优于WPSS(P=0.05),WPSS的预后优于IPSS(P=0.07)。老年患者IPSS-R优于IPSS和WPSS
The myelodysplastic syndromes (MDS) have highly variable outcomes and prognostic scoring systems are important tools for risk assessment and to guide therapeutic decisions. However, few population-based studies have compared the value of the different scoring systems. With data from the nationwide Swedish population-based MDS register we validated the International Prognostic Scoring System (IPSS), revised IPSS (IPSS-R) and the World Health Organization (WHO) Classification-based Prognostic Scoring System (WPSS). We also present population-based data on incidence, clinical characteristics including detailed cytogenetics and outcome from the register. The study encompassed 1329 patients reported to the register between 2009 and 2013, 14% of these had therapy-related MDS (t-MDS). Based on the MDS register, the yearly crude incidence of MDS in Sweden was 2.9 per 100000 inhabitants. IPSS-R had a significantly better prognostic power than IPSS (P < 0001). There was a trend for better prognostic power of IPSS-R compared to WPSS (P=0.05) and for WPSS compared to IPSS (P=0.07). IPSS-R was superior to both IPSS and WPSS for patients aged