越来越多的证据表明,在骨髓增生异常综合征的国际预后评分系统中低估了低风险细胞遗传学

Christian Steidl , Julie Schanz , Michael Pfeilstöcker , Thomas Nösslinger , Barbara Hildebrandt , Andrea Kuendgen , Michael Lübbert , Regina Kunzmann , Aristoteles Giagounidis , Carlo Aul , Lorenz Trümper , Otto Krieger , Reinhard Stauder , Thomas H. Müller , Friedrich Wimazal , Peter Valent , Christa Fonatsch , Ulrich Germing , Detlef Haase
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引用次数: 7

摘要

骨髓增生异常综合征(MDS)的国际预后评分系统(IPSS)将骨髓(BM)母细胞、细胞遗传学和细胞减少的数量确定为影响疾病结局的主要变量。这些变量的权重使用多变量分析方法定义不同的子组。最近的研究,包括我们小组的工作,表明IPSS中不利的细胞遗传学被低估了。为了描述低风险核型与细胞计数的预后影响,我们研究了1440名MDS患者的细胞遗传学和细胞计数亚组的临床结果。使用单变量分析工具,158例低风险细胞遗传学患者和66例blast计数为>20%分别为11.1个月和9个月。低风险细胞遗传学和正常细胞计数患者的中位生存时间(n = 60;中位生存期,17个月)与高危险核型和BM细胞高度升高的患者无显著差异(11%-20%:n = 89;中位生存期22个月;P = 0.098;比;20%: n = 32;中位生存期:13个月;P = .892)。我们的数据表明,低风险细胞遗传学与高度升高的骨髓母细胞相比具有同等的预后意义,并且表明在MDS的未来综合预后系统中,不利核型的评分更高。
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Growing Evidence for an Underestimation of Poor-Risk Cytogenetics in the International Prognostic Scoring System in Myelodysplastic Syndromes

The International Prognostic Scoring System (IPSS) for myelodysplastic syndromes (MDS) identifies bone marrow (BM) blasts, cytogenetics, and the number of cytopenias as major variables with impact on disease outcome. Weighting of these variables defines distinct subgroups using multivariate analysis approaches. Recent studies, including the work of our group, indicate an underestimation of unfavorable cytogenetics in the IPSS. To delineate the prognostic impact of poor-risk karyotypes in relation to blast counts, we studied clinical outcome of cytogenetic and blast count subgroups in a large cohort of 1440 patients with MDS. Using univariate analytic tools, median survival times of 158 patients with poor-risk cytogenetics and 66 patients with blast counts of > 20% were 11.1 months and 9 months, respectively. Median survival times of patients with poor-risk cytogenetics and normal blast counts (n = 60; median survival, 17 months) were not significantly different from those of good-risk karyotypes and highly elevated BM blasts (11%–20%: n = 89; median survival, 22 months; P = .098; > 20%: n = 32; median survival, 13 months; P = .892). Our data indicate an equal prognostic significance of poor-risk cytogenetics compared with highly elevated BM blasts and suggest a higher score for unfavorable karyotypes in future integrative prognostic systems in MDS.

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