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儿童Ph-like急性淋巴细胞白血病的研究进展

发布时间:2018-08-04 21:01
【摘要】: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。
[Abstract]:Philadelphia chromosome like acute lymphoblastic leukemia (Ph-like ALL) is a group of B-ALL (B-lineage ALL) subsets similar to Philadelphia chromosomal positive ALL (Ph~ ALL). It involves a series of cytokine receptor genes and related gene alterations associated with abnormal activation of kinase signaling pathway. The proportion of Ph-like ALL associated with lymphoid development was as high as 15% in high risk children with B-ALL. The clinical features were consistent with the poor prognosis. The combination of tyrosine kinase inhibitor (TKIs) and chemotherapy can significantly improve the prognosis of Ph- ALL in children. It is suggested that the precise targeting therapy based on Ph-like ALL molecular genetic abnormality has a good prospect. This article reviews the gene changes, pathogenesis, clinical features, diagnosis and treatment of Ph-like ALL in children.
【作者单位】: 四川大学华西第二医院儿科/出生缺陷与相关妇儿疾病教育部重点实验室;
【基金】:自然科学基金青年基金项目(81600122)
【分类号】:R733.71

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