How is the Ph-like signature being incorporated into ALL therapy?

How is the Ph-like signature being incorporated into ALL therapy?
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DOI:
10.1016/j.beha.2017.06.001
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发表时间:
2017-09-01
影响因子:
2.1
通讯作者:
Raetz, Elizabeth A.
Raetz, Elizabeth A.
中科院分区:
医学4区
文献类型:
--
作者:
Maese, Luke;Tasian, Sarah K.;Raetz, Elizabeth A.

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费城染色体样急性淋巴细胞白血病(Ph样ALL)是最近发现的一种高危疾病亚型,其特征是基因表达谱与费城染色体阳性(Ph阳性)ALL相似,但没有潜在的BCR-ABL 1易位。患有Ph样ALL的成人和儿童具有多种改变,所有这些改变都导致激活的激酶信号传导。Ph样ALL患者的结局较差,这促使研究基于酪氨酸激酶抑制剂(TKI)的治疗对这种疾病的作用。目前正在进行几项临床试验,包括筛查Ph样特征和用TKI治疗Ph样ALL患者。本综述探讨了如何将Ph样ALL检测纳入临床试验。(C)2017由Elsevier Ltd.出版
Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL) is a recently identified high risk disease subtype characterized by a gene expression profile similar to that observed in Philadelphia chromosome-positive (Ph-positive) ALL, but without an underlying BCR-ABL1 translocation. Adults and children with Ph-like ALL harbor a diversity of alterations that all lead to activated kinase signaling. Outcomes for patients with Ph-like ALL are poor, which has prompted investigation into the role of tyrosine kinase inhibitor (TKI)-based therapies for this disease. Several clinical trials are now ongoing that include screening for the Ph-like signature and treatment of patients with Ph-like ALL with TKI therapy. This review examines how testing for Ph-like ALL is being incorporated into clinical trials. (C) 2017 Published by Elsevier Ltd.