Tang Xue, Guo Xia
Department of Pediatrics, West China Second University Hospital, Sichuan University/Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610041, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2017 Nov;19(11):1213-1218. doi: 10.7499/j.issn.1008-8830.2017.11.017.
Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL) is a subtype of B-lineage ALL (B-ALL) that displays a gene expression profile (GEP) similar to Philadelphia chromosome-positive ALL (Ph ALL). It has a diverse range of genetic alterations that activate cytokine receptor genes and kinase signaling pathways, frequently accompanied by abnormal transcription factors related to lymphatic development. Children with Ph-like ALL account for 15% of children with high-risk B-ALL. It has adverse clinical features and a poor prognosis. Tyrosine kinase inhibitors combined with chemotherapy can significantly improve the prognosis of children with Ph ALL, suggesting that targeted therapy based on the molecular cytogenetic abnormalities of Ph-like ALL has good research prospects. This paper expounds the genetic alterations, pathogenesis, clinical features, diagnostic measures, and potential therapeutic approaches of Ph-like childhood ALL based on recent research progress in Ph-like ALL.
费城染色体样急性淋巴细胞白血病(Ph样ALL)是B系急性淋巴细胞白血病(B-ALL)的一种亚型,其基因表达谱(GEP)与费城染色体阳性急性淋巴细胞白血病(Ph ALL)相似。它具有多种激活细胞因子受体基因和激酶信号通路的基因改变,常伴有与淋巴发育相关的异常转录因子。Ph样ALL患儿占高危B-ALL患儿的15%。它具有不良的临床特征和较差的预后。酪氨酸激酶抑制剂联合化疗可显著改善Ph ALL患儿的预后,这表明基于Ph样ALL分子细胞遗传学异常的靶向治疗具有良好的研究前景。本文基于Ph样ALL的最新研究进展,阐述了儿童Ph样ALL的基因改变、发病机制、临床特征、诊断方法及潜在治疗方法。