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[原发性骨髓增生异常综合征患者不同细胞遗传学风险类别的预后意义研究]

[Study on prognostic significances of different cytogenetic risk categories in patients with primary myelodysplastic syndromes].

作者信息

Qu Shi-qiang, Liu Xu-ping, Xu Ze-feng, Zhang Yue, Qin Tie-jun, Zhang Tian-jiao, Cui Rui, Hao Yu-shu, Xiao Zhi-jian

机构信息

Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, State Key Laboratory of Experimental Hematology, Tianjin 300020, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2011 Dec;32(12):819-24.

Abstract

OBJECTIVE

To analyze significances of different cytogenetic categories for prognostic stratification in patients with primary myelodysplastic syndromes (MDS).

METHODS

Chromosomal abnormalities of 532 primary MDS patients were categorized according to cytogenetic categories of International Prognostic Scoring System (IPSS), Revised IPSS (IPSS-R), and German-Austrian (G-A). Prognostic impacts of different cytogenetic categories and frequent isolated anomalies were investigated.

RESULTS

Of 532 patients, 346(65%) patients had clonal cytogenetic abnormalities, including 200(38%) patients had 1 abnormality, 61(11%) patients had 2 abnormalities, and 85(16%) patients had complex abnormalities. Trisomy 8 was the most frequent karyotype abnormality, occurring in 31% of the patients with clonal cytogenetic abnormalities, other frequent anomalies were -7/del(7q)(13%), del(20q)(12%), del(5q)(9%), -18(5%), -21(5%), i(17q)(5%), -Y(4%), -17(4%), +21(4%), -13/del(13q)(4%), and -22(4%). The proportion of poor karyotypes of IPSS was higher in RAEBI and RAEBII among the World Health Organization classifications than in subgroups with less than 5% blasts. The follow-up data were available for 310 patients with a median follow-up duration of 14.5 months. Median survival was 59 months for patients with normal karyotypes and 26 months for those with abnormal karyotypes. According to IPSS cytogenetic categories, the median survivals of good-risk subgroup, intermediate-risk subgroup and poor-risk subgroup were 59, 43 and 12 months, respectively (P < 0.01). For IPSS-R cytogenetic groups, the median survivals of good-risk subgroup, intermediate-risk(int-risk) subgroup, poor-risk and very poor-risk subgroup were 59, 36, 15, and 10 months, respectively (P < 0.01). According to G-A classification, the median survivals of good-risk subgroup, int-1-risk subgroup, int-2-risk subgroup and poor-risk subgroup were 59, 44, 15, and 11 months, respectively (P < 0.01). In frequent isolated karyotypic abnormalities, +8 had a median survival of 44 months, i(17q) had a median survival of 12 months, and -7/del(7q) had a median survival of 14 months.

CONCLUSION

In comparison with IPSS and G-A categories, IPSS-R cytogenetic categories are more sophisticated, and can stratify prognosis effectively, but prognostic significances of some karyotypes in IPSS-R still need to be confirmed.

摘要

目的

分析不同细胞遗传学类别对原发性骨髓增生异常综合征(MDS)患者预后分层的意义。

方法

根据国际预后评分系统(IPSS)、修订的IPSS(IPSS-R)和德奥(G-A)细胞遗传学类别,对532例原发性MDS患者的染色体异常进行分类。研究不同细胞遗传学类别和常见孤立异常的预后影响。

结果

532例患者中,346例(65%)有克隆性细胞遗传学异常,其中200例(38%)有1种异常,61例(11%)有2种异常,85例(16%)有复杂异常。三体8是最常见的核型异常,在克隆性细胞遗传学异常患者中占31%,其他常见异常为-7/del(7q)(13%)、del(20q)(12%)、del(5q)(9%)、-18(5%)、-21(5%)、i(17q)(5%)、-Y(4%)、-17(4%)、+21(4%)、-13/del(13q)(4%)和-22(4%)。在世界卫生组织分类中,RAEB1和RAEB2中IPSS不良核型的比例高于原始细胞小于5%的亚组。310例患者有随访数据,中位随访时间为14.5个月。核型正常患者的中位生存期为59个月,核型异常患者为26个月。根据IPSS细胞遗传学类别,低危亚组、中危亚组和高危亚组的中位生存期分别为59、43和12个月(P<0.01)。对于IPSS-R细胞遗传学分组,低危亚组、中危(int-risk)亚组、高危和极高危亚组的中位生存期分别为59、36、15和10个月(P<0.01)。根据G-A分类,低危亚组、int-1-risk亚组、int-2-risk亚组和高危亚组的中位生存期分别为59、44、15和11个月(P<

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