原发骨髓增生异常综合征患者IPSS及染色体核型的临床分析

Clinical study on IPSS and karytype in the patients with primary myelodysplastic syndrome

目的:比较原发性骨髓增生异常综合征(MDS)患者WHO(2001)分型与FAB分型的IPSS染色体核型分析及预后的相关性分析。方法:经FAB标准确诊的原发MDS的患者重新按WHO标准分型,对2种结果的IPSS及染色体异常与各亚型的关系进行分析。结果:按FAB分型各亚型的IPSS及染色体异常无显著性差异,按WHO分型的难治性细胞减少伴多系增生异常(RCMD)与难治性贫血(RA)患者染色体异常率有统计学意义(66.6%,41.7%,P<0.01),RAEB-2高危组比例明显高于RAEB-1组(25%,0%,P<0.01)。结论:原发MDS的WHO分型与FAB分型相比,前者与预后的相关性更好。

Objective：Compare the relationship among IPSS, karytype of two classifications （FAB and WHO） and prognosis of primary MDS. Method：The patients diagnosed as primary MDS according to the FAB classification were reclassified following the WHO criteria. We compared the relationship between two kinds of IPSS, karyotype aberration and subtypes. Result：According to the FAB classification, there had no significant difference in IPSS and karyotype aberration between the two subtypes. According to the WHO classification, there had significant difference in karyotype aberration between patients with RCMD and RA （66.6 % and 41.7%, respectively, P〈0. 01）. The number of RAEB-2 in high-risk group were higher than that of RAEB-1 （25% and 0%, ,respectively,P〈0. 001）. Conclusion：Comparing to the FAB classification of primary MDS, the WHO classification had a better correlation to the prognosis.