P2RY8-CRLF2重排的急性淋巴细胞白血病儿童的临床特征及预后分析  

Clinical Features and Prognosis of Acute Lymphoblastic Leukemia Children with P2RY8-CRLF2 Gene Rearrangement

作　　者：郑湧智 乐少华[1] 郑浩[1] 华雪玲[1] 陈再生[1] 郑灵[1] 陈彩[1] 李梅[1] 蔡春霞 杨景辉[1] 陈以乔[1] 高琴丽[1] 陈莹莹[1] 李健[1] 胡建达[1] ZHENG Yong-Zhi;LE Shao-Hua;ZHENG Hao;HUA Xue-Ling;CHEN Zai-Sheng;ZHENG Ling;CHEN Cai;LI Mei;CAI Chun-Xia;YANG Jing-Hui;CHEN Yi-Qiao;GAO Qin-Li;CHEN Ying-Ying;LI Jian;HU Jian-Da(Department of Pediatric Hematology,Fujian Institute of Hematology,Fujian Provincial Key Laboratory of Hematology,Fujian Medical University Union Hospital,Fuzhou 350001,Fujian Province,China)

机构地区：[1]福建医科大学附属协和医院小儿血液科,福建省血液病研究所,福建省血液病学重点实验室,福建福州350001

出　　处：《中国实验血液学杂志》2021年第2期311-315,共5页Journal of Experimental Hematology

基　　金：福建省血液医学中心建设项目[闽政办(2017)4号];国家临床重点专科建设项目[闽财指(2011)1006号];福建省临床重点专科建设项目[闽卫科教(2012)149号]。

摘　　要：目的:探讨P2RY8-CRLF2重排急性淋巴细胞白血病(ALL)儿童的临床特征及预后。方法:2016年1-12(B-ALL)患儿,根据有无P2RY8-CRLF2重排分为P2RY8-CRLF2重排组和P2RY8-CRLF2阴性组。P2RY8-CRLF2重排组患儿均接受CCLG-ALL 2008高危组(HR)方案治疗,P2RY8-CRLF2阴性组患儿则按临床危险度分型接受不同强度化疗。结果:共检出P2RY8-CRLF2重排B-ALL患儿5例(4.6%),男4例,女1例,初诊中位年龄4(2-6)岁,初诊白细胞计数中位数26.12(2.46-525.1)×109/L,3例为普通B细胞型,2例早期前B细胞型;1例46,XY,der(20)[22]/46,XY[2],4例为正常核型。P2RY8-CRLF2重排组中,诱导缓解治疗第33天有1例(20%)未达完全缓解,2例(40%)微小残留病≥1%;P2RY8-CRLF2阴性组中,诱导缓解治疗第33天均达完全缓解率,6例(5.8%)微小残留病≥1%。P2RY8-CRLF2重排组3年无事件生存率显著低于P2RY8-CRLF2阴性组(60.0%±21.9%vs 85.9%±3.9%)(P=0.049)。结论:伴有P2RY8-CRLF2融合基因阳性的ALL儿童早期治疗反应较差,预后不良,仍需要探索更有效的治疗方法。Objective:To investigate the clinical features and prognostic factors of acute lymphoblastic leukemia(ALL)children with P2 RY8-CRLF2 gene rearrangement.Methods:A total of 108 children with B-cell ALL(B-ALL)were diagnosed and systematically treated according to Chinese Children’s Leukemia Group(CCLG)-ALL 2008 in our hospital from January 2016 to December 2016.The 108 patients were divided into two groups according to the result of mutiplex polymerase chain reaction:group with P2 RY8-CRLF2 gene rearrangement and group without P2 RY8-CRLF2 gene rearrangement.The ALL children with P2 RY8-CRLF2 gene rearrangement were all treated by CCLG-ALL 2008 high-risk group(HR)regimens,and the ALL children in group without P2 RY8-CRLF2 gene rearrangement received different intensity chemotherapy according to clinical risk classification.Results:Five(4 male and 1 female)out of108 patients with B-ALL had P2 RY8-CRLF2 gene rearrangement.In the 5 B-ALL patients with P2 RY8-CRLF2 gene rearrangement,the median age of the was 4(2-6)years old and the median WBC count was 26.2(2.46-525.1)×109/L.These patients presented different immunophenotype,including 3 cases of common B-ALL and 2 cases of pre B-ALL.Four patients carried a normal karyotype and 1 patient carried 46,XY,der(20)[22]/46,XY[2].For the children with P2 RY8-CRLF2 gene rearrangement,1 patient(20%)could not achieve complete remission(CR),and minimal residual disease(MRD)of 2 patients(40%)was higher than 1%on day 33 of induction chemotherapy;while in group without P2 RY8-CRLF2 gene rearrangement,all the patient achieved CR,and MRD in 6 patients(5.8%)was higher than 1%on day 33 of induction chemotherapy.The 3 year event-free survival(EFS)of ALL children in group with P2 RY8-CRLF2 gene rearrangement was significantly lower than that in group without P2 RY8-CRLF2 gene rearrangement(60.0%±21.9%vs 85.9%±3.9%)(P=0.049).Conclusion:The early treatment response and prognosis of ALL children with P2 RY8-CRLF2 gene rearrangement are worse,and more effective protocol is needed for this

关 键 词：P2RY8-CRLF2重排 急性淋巴细胞白血病 儿童 预后 

分 类 号：R733.71[医药卫生—肿瘤]