摘要
目前基于疾病危险度的分层诊断和治疗，已使急性淋巴细胞白血病（ALL）的无病生存率和总生存率显著提高。但仍然有部分患者疗效不佳，易复发。随着基因测序技术的进展，越来越多难治复发ALL的分子遗传学异常被揭示。Ph样ALL是近年来新分类的一组基因表达谱与BCR—ABL1阳性ALL相似的亚群，涉及一系列细胞因子和激酶信号通路活化相关的分子异常。其发病率高、预后差，但联合酪氨酸激酶抑制剂（TKI）治疗有望提高其生存和预后。在2014年的第56届美国血液学会（ASH）年会上，Ph样ALL仍然是一个热点话题。文章结合近年来Ph样ALL的研究进展、此次会议的报告内容以及作者所在医院的工作经验对其进行介绍。
The event free survival and overall survival rate of acute lymphoblastic leukemia （ALL） have been improved significantly based on the risk stratify diagnosis and treatment. But there are still some patients suffering therapeutic failure and relapse. With the great development of genome sequencing technology, more and more genetic aberrations behind the refractory and relapse ALL have been identified. Ph-like ALL is charactered with gene expression profile similar to that of BCR-ABL1 positive ALL, involving abnormal activation of cytokine receptor and tyrosine kinase. It has been proved that treatment combined with Tyrosine kinase inhibitors （TKIs） can significantly improve the poor prognosis. Ph-like ALL is one of hot topics during the 56th American Society of Hematology （ASH） annual meeting in 2014. Progression in molecular genetics for Ph-like ALL will be introduced together with the author＇s research experience.
出处
《白血病．淋巴瘤》
CAS
2015年第2期74-78,共5页
Journal of Leukemia & Lymphoma
关键词
Ph样急性淋巴细胞白血病
酪氨酸激酶抑制剂
美国血液学会年会
Ph-like acute lymphoblastic leukemia
Tyrosine kinase inhibitors
American Society of Hematology annual meeting
