Pathogenesis of ETV6/RUNX1-positive childhood acute lymphoblastic leukemia and mechanisms underlying its relapse.
Pathogenesis of ETV6/RUNX1-positive childhood acute lymphoblastic leukemia and mechanisms underlying its relapse.
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ETV6/RUNX1阳性儿童急性淋巴细胞白血病的发病机制及其复发机制
DOI：
10.18632/oncotarget.16367
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发表时间：
2017-05-23
期刊：
影响因子：
--
通讯作者：
Zhu X
中科院分区：
文献类型：
--
作者：
Sun C;Chang L;Zhu X
ETV 6/RUNX 1（E/R）是儿童急性淋巴细胞白血病（ALL）中最常见的融合基因。多条证据暗示了E/R阳性ALL的分子发病机制的“两次打击”模型，即E/R重排后发生一系列继发性突变，触发明显的白血病。E/R产生的细胞框架和E/R对白血病前状态的维持是白血病发生机制的基础。因此，各种研究都集中在引起原发性和复发性E/R阳性ALL的克隆之间的关系。我们在这里回顾了最新的见解，潜在的E/R阳性ALL的致病机制，以及复发时普遍存在的分子异常。
ETV6/RUNX1 (E/R) is the most common fusion gene in childhood acute lymphoblastic leukemia (ALL). Multiple lines of evidence imply a “two-hit” model for the molecular pathogenesis of E/R-positive ALL, whereby E/R rearrangement is followed by a series of secondary mutations that trigger overt leukemia. The cellular framework in which E/R arises and the maintenance of a pre-leukemic condition by E/R are fundamental to the mechanism that underlies leukemogenesis. Accordingly, a variety of studies have focused on the relationship between the clones giving rise to the primary and recurrent E/R-positive ALL. We review here the most recent insights into the pathogenic mechanisms underlying E/R-positive ALL, as well as the molecular abnormalities prevailing at relapse.