Division of Blood and Marrow Transplantation, Stanford University, Stanford, CA 94305, USA.
Department of Pathology and Human Anatomy, Loma Linda University, Loma Linda, CA 92350, USA.
Int J Mol Sci. 2020 Mar 22;21(6):2193. doi: 10.3390/ijms21062193.
Philadelphia (Ph)-like acute lymphoblastic leukemia (ALL) is a high-risk B-cell Acute Lymphoblastic Leukemia (B-ALL) characterized by a gene expression profile similar to Ph-positive B-ALL but lacking the translocation. The molecular pathogenesis of Ph-like B-ALL is heterogenous and involves aberrant genomics, receptor overexpression, kinase fusions, and mutations leading to kinase signaling activation, leukemogenic cellular proliferation, and differentiation blockade. Testing for the Ph-like signature, once only a research technique, is now available to the clinical oncologist. The plethora of data pointing to poor outcomes for this ALL subset has triggered investigations into the role of targeted therapies, predominantly involving tyrosine kinase inhibitors that are showing promising results.
费城（Ph）样急性淋巴细胞白血病（ALL）是一种高风险 B 细胞急性淋巴细胞白血病（B-ALL），其特征是基因表达谱类似于 Ph 阳性 B-ALL，但缺乏易位。Ph 样 B-ALL 的分子发病机制是异质性的，涉及异常基因组学、受体过表达、激酶融合以及导致激酶信号激活、白血病细胞增殖和分化阻滞的突变。Ph 样特征的检测，曾经只是一种研究技术，现在已经可以提供给临床肿瘤学家。大量的数据表明，这种 ALL 亚组的预后较差，这促使人们研究靶向治疗的作用，主要涉及酪氨酸激酶抑制剂，这些抑制剂显示出有希望的结果。
