费城样急性淋巴细胞白血病的诊疗进展  

Advances in the Diagnosis and Treatment of Ph-like Acute Lymphoblastic Leukemia

作　　者：王玉[1] 于洪娟[1] 吕成芳[1] WANG Yu;YU Hongjuan;LYU Chengfang(Department of Hematology,the First Affiliated Hospital of Harbin Medical University,Harbin 150001,China)

机构地区：[1]哈尔滨医科大学附属第一医院血液内科

出　　处：《医学综述》2019年第16期3164-3169,共6页Medical Recapitulate

基　　金：黑龙江省博士后资助经费项目(LBH-Z16240)

摘　　要：费城样急性淋巴细胞白血病(Ph-likeALL)是一种新提出的高危急性B淋巴细胞白血病亚型,具有与费城染色体阳性急性淋巴细胞白血病相似的基因表达谱,与不良临床特征相关,且预后差。该亚型的筛查及诊断方法尚未标准化,二代测序是目前最可靠的检测方法。Ph-likeALL具有激活细胞因子受体基因和激酶信号转导途径的广泛遗传学改变的特点,使其适于用酪氨酸激酶抑制剂(TKIs)治疗,但仍需要多中心、大规模的前瞻性试验证实TKIs联合化疗的疗效及对预后的影响。此外,针对TKIs耐药的改善方法及治疗Ph-likeALL的新策略尚在临床前研究中。Philadelphia chromosome-like acute lymphoblastic leukemia(Ph-like ALL) is a recently identified subtype of high-risk B-cell acute lymphoblastic leukemia with a gene expression profile similar to that observed in Philadelphia chromosome -positive acute lymphoblastic leukemia,it is associated with adverse clinical features and poor outcome.The screening and diagnostic methods for Ph-like ALL is still not standardized.Next generation sequencing is considered to be the most reliable and available detection method at present. It harbors a diverse range of genetic alterations that activate cytokine receptor genes and kinase signaling pathways,making it amenable to treatment with Tyrosine kinase inhibitors (TKIs) therapy .But multicenter and large-scale prospective trials are still needed to confirm the efficacy and impact on prognosis of TKIs in combination with chemotherapy.In addition,improved methods for TKIs resistance and new strategies for the treatment of Ph-like ALL are still in preclinical studies.

关 键 词：费城样急性淋巴细胞白血病 基因 诊断 酪氨酸激酶抑制剂 

分 类 号：R551[医药卫生—血液循环系统疾病]