Xu Jing-Jing, Xiong Hui-Xia
Graduate School of Qinghai University, Xining 810001, Qinghai Province, China.Gaochun People's Hospital, Nanjing 211300, Jiangsu Province, China.
Department of Hematology, Affiliated Hospital of Qinghai University, Xining 810001, Qinghai Province, China.E-mail: xhx-73@163. com.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2018 Oct;26(5):1579-1582. doi: 10.7534/j.issn.1009-2137.2018.05.054.
Ph-like acute lymphoblastic leukemia (ALL) is a high-risk subtype of precursor B-cell acute lymphoblastic leukemia (BCP-ALL) with a gene expression profile and a high frequency of IKZF1 gene alteration similar to that of Ph-positive ALL, which is a clinically and biologically heterogeneous subtype of BCP-ALL. The prognosis correlats negatively with age increasing. The incidence of this "Ph-like" subtype may be higher in young adults. Ph-like ALL is characterized by genetic alterations that activate cytokine receptor genes and kinase signaling pathways. Prospective studies are needed to determine if incorporation of tyrosine kinase inhibitor-targeting kinase alterations into intensive chemotherapy regimens will improve outcome of patients with Ph-like ALL. The research progress of Ph-like ALL is summarised in this review, including epidemiology, genetic alterations and treatment..
Ph样急性淋巴细胞白血病（ALL）是前体B细胞急性淋巴细胞白血病（BCP-ALL）的一种高危亚型，其基因表达谱以及IKZF1基因改变的频率与Ph阳性ALL相似，后者是BCP-ALL的一种临床和生物学上异质性的亚型。预后与年龄增长呈负相关。这种“Ph样”亚型在年轻成年人中的发病率可能更高。Ph样ALL的特征是激活细胞因子受体基因和激酶信号通路的基因改变。需要进行前瞻性研究，以确定将靶向激酶改变的酪氨酸激酶抑制剂纳入强化化疗方案是否会改善Ph样ALL患者的预后。本文综述了Ph样ALL的研究进展，包括流行病学、基因改变和治疗。
