[The International Prognostic Scoring System for primary myelodysplastic syndrome].

Sbornik lekarsky Pub Date : 2002-01-01
M Sisková, A Dohnalová, R Neuwirtová, J Pĕnicková, J Karban, E Cmunt
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Abstract

A number of prognostic scoring systems for patients with myelodysplastic syndrome (MDS) have been introduced since FAB classification of the MDS in 1982. Recently, the International Prognostic Scoring System (IPSS), published in 1997 by Greenberg et al. [9] is based on the percentage of bone marrow (BM) blasts, cytogenetic abnormalities and number of cytopenias. We applied criteria of the IPSS on 205 patients (pts) with primary MDS (RA = 82, RARS = 49, RAEB = 42, RAEB-t = 8, CMML = 24 pts). IPSS discriminated within each of the FAB-subgroups: RA pts were present in low risk and intermediate (Int) I and II risk subgroups, RARS pts were separated into low and Int I, RAEB were distributed predominantly between Int I and Int II risk groups, RAEB-t in high-risk group, and CMML pts were distributed in all groups. In contrary to Greenberg's group of the MDS patients there are only three risk-groups in our study: low risk (score 0-0.5), intermediate (1-2) and high risk (> 2); the median survival and the risk of the evolution to the acute leukemia (p = 0.0001) are significantly different.

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[原发性骨髓增生异常综合征的国际预后评分系统]。
自1982年骨髓增生异常综合征(MDS)的FAB分类以来,已经引入了许多用于MDS患者的预后评分系统。最近,Greenberg等人[9]于1997年发表的国际预后评分系统(International Prognostic Scoring System, IPSS)是基于骨髓(BM)原细胞百分比、细胞遗传学异常和细胞减少数量。我们将IPSS标准应用于205例原发性MDS患者(RA = 82, RARS = 49, RAEB = 42, RAEB-t = 8, CMML = 24)。IPSS在各fab亚组中均有区别:RA患者存在于低危、中危(Int) I和II风险亚组,RARS患者分为低危和I危,RAEB主要分布于I危和II危组,RAEB-t主要分布于高危组,CMML患者分布于所有组。与Greenberg的MDS患者组相反,我们的研究中只有三个风险组:低风险(0-0.5分)、中风险(1-2分)和高风险(> 2分);中位生存期和发展为急性白血病的风险差异有统计学意义(p = 0.0001)。
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