急性B淋巴细胞白血病发病机制研究进展

发布时间：2018-04-28 22:03

本文选题：B-ALL + 融合基因　；参考：《中国科学:生命科学》2017年12期

【摘要】：急性B淋巴细胞白血病(B-ALL)是由于B淋巴细胞发育和增殖相关基因等突变,阻碍了B淋巴祖细胞的正常分化和成熟,伴随着异常增殖而引起的克隆异质性疾病,是高发于儿童的一种主要血液系统恶性肿瘤.基于对已有基因突变导致的B-ALL发病机制的深入理解和研究,B-ALL的治愈率在儿童患者中达80%以上,但仍有约20%的儿童患者和60%的成人患者最终复发.近年来,利用基因芯片和二代测序等技术在复发的B-ALL患者检测到很多新的基因突变类型,但是这些基因突变与其他协同融合基因在B-ALL中的的具体发病机制仍不明确.本文主要从涉及细胞发育分化、表观遗传调控、细胞周期和凋亡、细胞信号转导通路、非编码RNA等协同基因突变以及白血病干细胞方面分析B-ALL发生和耐药复发的机制,并总结分析目前常用的B-ALL相关小鼠(Mus musculus)模型,以期对复发性B-ALL的发病机制、风险分级和治疗策略等给予全新的认识.
[Abstract]:Acute B lymphoblastic leukemia (B-ALL) is a clonal heterogeneity disease caused by the mutation of B lymphocyte development and proliferation-related genes, which hinders the normal differentiation and maturation of B lymphoid progenitor cells, and is accompanied by abnormal proliferation. It is a major hematological malignancy in children. Based on the deep understanding and study of the pathogenesis of B-ALL caused by gene mutation, the cure rate of BALL is more than 80% in children, but there are still about 20% of children and 60% of adult patients relapse. In recent years, gene chip and second-generation sequencing techniques have been used to detect many new gene mutations in recurrent B-ALL patients, but the specific pathogenesis of these mutations and other co-fusion genes in B-ALL is still unclear. In this paper, the mechanisms of B-ALL development and drug resistance recurrence were analyzed from the aspects of cell development and differentiation, epigenetic regulation, cell cycle and apoptosis, cell signal transduction pathway, co-gene mutations such as non-coding RNA and leukemic stem cells. The present model of Mus musculus associated with B-ALL was summarized and analyzed in order to give a new understanding of the pathogenesis, risk classification and treatment strategy of recurrent B-ALL.
【作者单位】：中国医学科学院&北京协和医学院血液病医院(血液学研究所)实验血液学国家重点实验室;中国医学科学院干细胞医学中心;
【基金】：国家自然科学基金(批准号:81470279,81500085,81670105) 中国医学科学院&北京协和医学院协和青年基金项目(批准号:332015127)资助
【分类号】：R733.71

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