TLS-ERG融合基因阳性急性髓系白血病9例并文献复习  

Acute myeloid leukemia with positive TLS-ERG fusion gene: report of 9 cases and review of literature

作　　者：韩梦媛 谭业辉[1] 胡瑞萍[1] 赵杨祉[1] 丁筱[1] 李昱瑛[1] 刘晓亮[1] 林海[1] 谢梦青 杨岩[1] 孙京男[1] 高素君[1] Han Mengyuan;Tan Yehui;Hu Ruiping;Zhao Yangzhi;Ding Xiao;Li Yuying;Liu Xiaoliang;Lin Hai;Xie Mengqing;Yang Yan;Sun Jingnan;Gao Sujun(Department of Hematology,the First Hospital of Jilin University,Changchun 130021,China)

机构地区：[1]吉林大学第一医院血液科,长春130021

出　　处：《白血病．淋巴瘤》2022年第10期603-605,共3页Journal of Leukemia & Lymphoma

基　　金：吉林省自然科学基金(20210101327JC)。

摘　　要：目的:探讨TLS-ERG融合基因阳性急性髓系白血病(AML)患者的临床特点及预后。方法:回顾性分析2013年6月至2020年8月在吉林大学第一医院诊治的9例TLS-ERG融合基因阳性AML患者的临床资料,并复习相关文献。结果:9例TLS-ERG融合基因阳性AML患者中,男性5例,女性4例,中位年龄16岁(6~40岁)。5例患者接受单纯化疗,3例患者接受异基因造血干细胞移植(allo-HSCT),1例患者未系统治疗。8例系统治疗患者中,首次诱导化疗完全缓解1例,诱导治疗完全缓解5例。5例单纯化疗患者中位总生存时间为1.5个月(1~11个月);其中3例首疗程未缓解,因感染死亡,2例复发后死亡。3例移植患者中位总生存时间为16个月(13~17个月);其中2例复发后死亡,1例随访截止前持续分子学完全缓解。结论:TLS-ERG融合基因阳性在AML中发生率低,诱导治疗效果差,造血干细胞移植可能改善部分患者的预后,但并不能克服TLS-ERG融合基因阳性对预后的不良影响。Objective:To investigate the clinical characteristics and prognosis of acute myeloid leukemia (AML) patients with positive TLS-ERG fusion gene.Methods:The clinical data of 9 AML patients with positive TLS-ERG fusion gene in the First Hospital of Jilin University from June 2013 to August 2020 were retrospectively analyzed, and the relevant literature was reviewed.Results:Among 9 patients with positive TLS-ERG fusion gene, there were 5 males and 4 females, with a median age of 16 years old (6-40 years old). Five patients received chemotherapy alone, 3 patients received allogeneic hematopoietic stem cell transplantation (allo-HSCT), and 1 patient did not receive systematic treatment. Among 8 patients with systematic treatment, 1 patient had complete remission after the first induction chemotherapy and 5 patients had complete remission after induction therapy. The median overall survival time of 5 patients with chemotherapy alone was 1.5 months (1-11 months), of which 3 patients did not respond to the first course of treatment and died of infection, and 2 patients died after relapse. The median overall survival time of 3 patients with allo-HSCT was 16 months (13-17 months), of which 2 patients died after relapse and 1 patient had sustained molecular complete remission by the end of follow-up.Conclusions:AML with positive TLS-ERG fusion gene has low incidence rate and poor induction efficacy. Hematopoietic stem cell transplantation may partially improve the survival prognosis of patients, but it cannot overcome the adverse effect of positive TLS-ERG fusion gene on prognosis.

关 键 词：白血病 髓样 急性基因融合预后TLS-ERG 

分 类 号：R733.71[医药卫生—肿瘤]