原发性骨髓增生异常综合征患者染色体核型与IPSS危度分组:FAB与WHO分型比较

Karyotypic and IPSS grouping of primary myelodysplastic syndromes patients: a comparison between FAB-and WHO-classification

目的 比较原发性骨髓增生异常综合征 (pMDS)患者WHO(2 0 0 1)分型和FAB分型的亚型与细胞遗传学异常及预后的相关性。方法 按FAB标准确诊并进行了染色体核型分析的 2 37例pMDS患者 ,重新进行血片和骨髓片分类计数并按WHO标准进行分型 ,对两种分型结果的染色体核型异常和国际预后积分系统 (IPSS)危度分组与各亚型的关系进行比较。结果 按FAB标准分型各亚型的染色体异常检出率及染色体异常危度分组无显著性差异 ,按WHO标准分型的难治性血细胞减少伴多系发育异常 (RCMD)患者与RA患者染色体核型异常检出率有显著性差异 (分别为 74 .4 %和 4 2 .5 % ,P <0 .0 0 1) ,IPSS预后好的染色体核型比例RA组 (6 5 .0 % )明显高于RCMD组 (2 4 .4 % )(P <0 .0 0 1) ,中等及差的染色体核型比例RCMD组 (分别为 4 8.9% ,2 6 .7% )明显高于RA组 (分别为 2 7.5 % ,7.5 % ) (P <0 .0 5 )。WHO分型与FAB分型一样 ,各亚型与IPSS危度分组有较好的相关性 ,WHO分型的RCMD低危组比例 (1.1% )较RA(10 .0 % )明显减低 (P <0 .0 5 ) ,RAEB Ⅱ高危组比例 (30 .5 % )较RAEB Ⅰ (0 )明显增高 (P <0 .0 0 1)。

Objective To compare the results of cytogenetic and IPSS grouping of primary myelodysplastic syndromes (pMDS) patients classified by FAB- or WHO classification. Methods Two hundred and thirty seven cases of pMDS who were previously classified according to FAB criteria were reclassified with WHO classification. A comparison was made between the results of the two classifications. Results For the detection rates of cytogenetic abnormality and its risks group, there was no difference among the FAB subgroups but the detection rate was different between the WHO refractory cytopenia with multilineage dysplasia (RCMD) and RA subgroups(74.4% and 42.5%,respectively)(P<0.001).The percentage of good karyotype abnormalities in RA(65.0%) was higher than that in RCMD(24.4%)(P<0.001), and the percentages of intermediate and poor karyotype abnormalities in RCMD(48.9% and 26.7%,respectively) were higher than that in RA(27.5% and 7.5%,respectively) (P<0.05).There was a good correlation between the subgroups and IPSS risk groups for both the WHO classification and the FAB classification, but the WHO classification further reflected the differences between RCMD and RA and RAEB-Ⅰand RAEB-Ⅱsubgroups. The percentage of low-risk group in RCMD(1.1%) was lower than that in RA(10.0%)(P<0.05),and the percentage of high-risk group in RAEB-Ⅱ(30.5%) was higher than that in RAEB-Ⅰ(0)(P<0.001). Conclusion For the correlation between subgroups and cytogenetic abnormalities and IPSS risk groups,the WHO-classification is better than the FAB-classification.