【摘要】：費(fèi)城染色體樣急性淋巴細(xì)胞白血病(Ph-like ALL)是一組基因表達(dá)譜與費(fèi)城染色體陽(yáng)性ALL(Ph~+ALL)相似的B-ALL(B-lineage ALL)亞群,涉及一系列細(xì)胞因子受體基因及激酶信號(hào)通路異�；罨南嚓P(guān)基因改變,并常伴淋系發(fā)育相關(guān)轉(zhuǎn)錄因子異常。Ph-like ALL在高危組兒童B-ALL的比例高達(dá)15%,其臨床特征與不良預(yù)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(yù)后提示基于Ph-like ALL分子遺傳學(xué)異常的精準(zhǔn)靶向治療具有良好的研究前景。該文結(jié)合近年P(guān)h-like ALL的相關(guān)研究進(jìn)展,對(duì)兒童Ph-like ALL的基因改變及發(fā)病機(jī)制、臨床特征、診斷及治療進(jìn)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(xué)華西第二醫(yī)院兒科/出生缺陷與相關(guān)婦兒疾病教育部重點(diǎn)實(shí)驗(yàn)室;
【基金】：自然科學(xué)基金青年基金項(xiàng)目(81600122)
【分類(lèi)號(hào)】：R733.71

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