【摘要】：費城染色體樣急性淋巴細胞白血病(Ph-like ALL)是一組基因表達譜與費城染色體陽性ALL(Ph~+ALL)相似的B-ALL(B-lineage ALL)亞群,涉及一系列細胞因子受體基因及激酶信號通路異常活化的相關基因改變,并常伴淋系發(fā)育相關轉(zhuǎn)錄因子異常。Ph-like ALL在高危組兒童B-ALL的比例高達15%,其臨床特征與不良預后相一致。酪氨酸激酶抑制劑(TKIs)聯(lián)合化療顯著改善兒童Ph~+ALL預后提示基于Ph-like ALL分子遺傳學異常的精準靶向治療具有良好的研究前景。該文結(jié)合近年Ph-like ALL的相關研究進展,對兒童Ph-like ALL的基因改變及發(fā)病機制、臨床特征、診斷及治療進行綜述。
[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.
【作者單位】： 四川大學華西第二醫(yī)院兒科/出生缺陷與相關婦兒疾病教育部重點實驗室;
【基金】：自然科學基金青年基金項目(81600122)
【分類號】：R733.71

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