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费城染色体样急性淋巴细胞白血病:一种新型癌症亚型的研究进展

Philadelphia chromosome-like acute lymphoblastic leukemia: progress in a new cancer subtype.

作者信息

Wells Julia, Jain Nitin, Konopleva Marina

机构信息

Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.

出版信息

Clin Adv Hematol Oncol. 2017 Jul;15(7):554-561.

Abstract

Philadelphia chromosome-like (Ph-like) acute lymphoblastic leukemia (ALL) is a newly described, high-risk subtype of B-cell ALL. It is characterized by a gene expression profile similar to that of Ph-positive ALL; however, the BCR-ABL1 fusion is not present. The World Health Organization classification of myeloid neoplasms and acute leukemia recently was updated to include the Ph-like or BCR-ABL1-like ALL subtype of B-cell ALL as a provisional entity. Unlike Ph-positive ALL, which is characterized by the pathognomonic BCR-ABL1 fusion, Ph-like ALL is characterized by a multitude of different genetic rearrangements and mutations. In this review, we outline the age-related and geographic incidence of Ph-like ALL, the association with worse clinical outcomes, and early evidence for the use of ruxolitinib (a Janus kinase 2 inhibitor) and dasatinib (a tyrosine kinase inhibitor targeting ABL1).

摘要

费城染色体样(Ph样)急性淋巴细胞白血病(ALL)是一种新描述的高危B细胞ALL亚型。其特征是基因表达谱与Ph阳性ALL相似;然而,不存在BCR-ABL1融合。世界卫生组织对髓系肿瘤和急性白血病的分类最近进行了更新,将B细胞ALL的Ph样或BCR-ABL1样ALL亚型作为一个临时实体纳入其中。与以特征性BCR-ABL1融合为特征的Ph阳性ALL不同,Ph样ALL的特征是多种不同的基因重排和突变。在本综述中,我们概述了Ph样ALL的年龄相关和地理发病率、与较差临床结局的关联,以及使用鲁索替尼(一种Janus激酶2抑制剂)和达沙替尼(一种靶向ABL1的酪氨酸激酶抑制剂)的早期证据。

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