Wells Julia, Jain Nitin, Konopleva Marina
Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.
Clin Adv Hematol Oncol. 2017 Jul;15(7):554-561.
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).
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。