儿童Ph-like急性淋巴细胞白血病的研究进展

费城染色体样急性淋巴细胞白血病（Ph-like ALL）是一组基因表达谱与费城染色体阳性ALL（Ph~＋ALL）相似的B-ALL（B-lineage ALL）亚群,涉及一系列细胞因子受体基因及激酶信号通路异常活化的相关基因改变,并常伴淋系发育相关转录因子异常。Ph-like ALL在高危组儿童B-ALL的比例高达15%,其临床特征与不良预后相一致。酪氨酸激酶抑制剂（TKIs）联合化疗显著改善儿童Ph~＋ALL预后提示基于Ph-like ALL分子遗传学异常的精准靶向治疗具有良好的研究前景。该文结合近年Ph-like ALL的相关研究进展,对儿童Ph-like ALL的基因改变及发病机制、临床特...

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Bibliographic Details
Published inZhongguo dang dai er ke za zhi Vol. 19; no. 11; pp. 1213 - 1218
Main Author 唐雪 郭霞
Format Journal Article
LanguageChinese
English
Published 中国长沙 中国当代儿科杂志编辑部 25.11.2017
Subjects
Ph-like急性淋巴细胞白血病
Review
儿童
基因
综述
诊断
酪氨酸激酶抑制剂
Online AccessGet full text
ISSN1008-8830
DOI10.7499/j.issn.1008-8830.2017.11.017

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Summary:费城染色体样急性淋巴细胞白血病（Ph-like ALL）是一组基因表达谱与费城染色体阳性ALL（Ph~＋ALL）相似的B-ALL（B-lineage ALL）亚群,涉及一系列细胞因子受体基因及激酶信号通路异常活化的相关基因改变,并常伴淋系发育相关转录因子异常。Ph-like ALL在高危组儿童B-ALL的比例高达15%,其临床特征与不良预后相一致。酪氨酸激酶抑制剂（TKIs）联合化疗显著改善儿童Ph~＋ALL预后提示基于Ph-like ALL分子遗传学异常的精准靶向治疗具有良好的研究前景。该文结合近年Ph-like ALL的相关研究进展,对儿童Ph-like ALL的基因改变及发病机制、临床特征、诊断及治疗进行综述。
Bibliography:43-1301/R
TANG Xue, GUO Xia. （Department of Pediatrics, West China Second University Hospital, Sichuan University~Key Laboratory of Birth Defects and Related Diseases of Women and Children （Sichuan University）, Ministry of Education, Chengdu 610041, China ）
Ph-like acute lymphoblastic leukemia; Gene; Diagnosis; Tyrosine kinase inhibitor; Child
Philadelphia chromosome-like acute lymphoblastic leukemia（Ph-like ALL） is a subtype of B-lineage ALL（B-ALL） that displays a gene expression profile（GEP） similar to Philadelphia chromosome-positive ALL（Ph~＋ALL）.It has a diverse range of genetic alterations that activate cytokine receptor genes and kinase signaling pathways,frequently accompanied by abnormal transcription factors related to lymphatic development.Children with Ph-like ALL account for 15% of children with high-risk B-ALL.It has adverse clinical features and a poor prognosis.Tyrosine kinase inhibitors combined with chemotherapy can significantly improve the prognosis of children with Ph＋ ALL,suggesting that tar
ISSN:1008-8830
DOI:10.7499/j.issn.1008-8830.2017.11.017