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PAX5缺失对于儿童无特殊重现染色体异常B系急性淋巴细胞白血病的预后意义

刘晓明 杨文钰 郭晔 邹尧 陈玉梅 竺晓凡 张丽 阮敏 刘天峰 张家源 刘芳 戚本泉 陈晓娟 王书春

中国当代儿科杂志2016,Vol.18Issue(4):287-291,5.
中国当代儿科杂志2016,Vol.18Issue(4):287-291,5.DOI:10.7499/j.issn.1008-8830.2016.04.001

PAX5缺失对于儿童无特殊重现染色体异常B系急性淋巴细胞白血病的预后意义

Signiifcance of PAX5 deletion in childhood B-lineage acute lymphoblastic leukemia without reproducible chromosomal abnormalities

刘晓明 1杨文钰 1郭晔 1邹尧 1陈玉梅 1竺晓凡 1张丽 1阮敏 1刘天峰 1张家源 1刘芳 1戚本泉 1陈晓娟 1王书春1

作者信息

  • 1. 中国医学科学院北京协和医学院/血液病医院血液学研究所/儿童血液病诊疗中心,天津 300020
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摘要

Abstract

ObjectiveTo identify the incidence of PAX5 deletion in childhood B-lineage acute lymphoblastic leukemia (B-ALL) without reproducible chromosomal abnormalities and to investigate the association between PAX5 abnormalities and prognosis of ALL.MethodsMultiplex ligation-dependent probe ampliifcation was used to determine the copy numbers of PAX5 gene in children newly diagnosed with B-ALL without reproducible chromosomal abnormalities between April 2008 and April 2013 and controls (children with non-hematologic diseases or tumors). The patients were classiified into deletion group and non-deletion group based on the presence of PAX5 deletion.Results Eighteen (21%) out of 86 children with B-ALL had PAX5 deletion. The deletion group had a signiifcantly higher total white blood cell count at diagnosis than the non-deletion group (P=0.001). The Kaplan-Meier analysis demonstrated that the deletion group had a signiifcantly lower disease-free survival (DFS) rate than the non-deletion group (0.69±0.12 vs 0.90±0.04;P=0.017), but there was no signiifcant difference in the overall survival rate between the two groups (P=0.128). The Cox analysis showed that PAX5 deletion was a risk factor for DFS (P=0.03).ConclusionsPAX5 deletion is an independent risk factor for DFS in B-ALL children without reproducible chromosomal abnormalities.

关键词

PAX5/B-急性淋巴细胞白血病/多重连接探针扩增/预后/儿童

Key words

PAX5/B-lineage acute lymphoblastic leukemia/Multiplex ligation-dependent probe ampliifcation/Prognosis/Child

引用本文复制引用

刘晓明,杨文钰,郭晔,邹尧,陈玉梅,竺晓凡,张丽,阮敏,刘天峰,张家源,刘芳,戚本泉,陈晓娟,王书春..PAX5缺失对于儿童无特殊重现染色体异常B系急性淋巴细胞白血病的预后意义[J].中国当代儿科杂志,2016,18(4):287-291,5.

基金项目

天津市科技支撑计划(12ZCDZSY18100);国家自然科学基金项目(81470339,81170470)。 ()

中国当代儿科杂志

OA北大核心CSCDCSTPCDMEDLINE

1008-8830

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