伴SET-NUP214融合基因急性白血病异基因造血干细胞移植疗效分析  

Allogeneic hematopoietic stem cell transplantation in acute leukemia patients with the SET-NUP214 fusion gene: Efficacy and survival analysis

作　　者：夏晶[1,2] 赵晔[1,2] 陈峰[1,2] 苗瞄[1] 仇惠英[1] 马骁[1,2] 唐晓文[1] 王荧[1] 吴小津[1,2] 傅铮铮[1] 吴德沛[1] 陈苏宁[1] Xia Jing;Zhao Ye;Chen Feng;Miao Miao;Qiu Huiying;Ma Xiao;Tang Xiaowen;Wang Ying;Wu Xiaojin;Fu Zhengzheng;Wu Depei;Chen Suning(Department of Hematology,the First Affiliated Hospital of Soochow University,National Clinical Research Center for Hematologic Diseases,Jiangsu Institute of Hematology,Suzhou 215006,China;the First Affiliated Hospital of Soochow University,Soochow Hongci Hematology Hospital,Suzhou 215100,China)

机构地区：[1]苏州大学附属第一医院血液科,国家血液系统疾病临床医学研究中心,江苏省血液研究所,苏州215006 [2]苏州大学附属第一医院,苏州弘慈血液病医院,苏州215100

出　　处：《中华内科杂志》2023年第4期410-415,共6页Chinese Journal of Internal Medicine

基　　金：国家重点研发计划(2019YFC0840604);江苏省重点研发计划(BE2019798);江苏省科教强卫工程-临床医学中心(YXZXA2016002)。

摘　　要：目的:探讨异基因造血干细胞移植(allo-HSCT)治疗伴SET-NUP214融合基因急性白血病(SET-NUP214+AL)的疗效。方法:回顾性病例系列研究。收集2014年12月至2021年10月在苏州大学附属第一医院和苏州弘慈血液病医院接受allo-HSCT治疗的18例SET-NUP214+AL患者的临床资料,分析其疗效及预后,采用Kaplan-Meier法进行生存分析。结果:18例患者中男性12例,女性6例,年龄13~55岁,中位数29岁。急性混合细胞白血病6例(髓/T表达3例、B/T表达2例、髓/B/T表达1例),急性淋巴细胞白血病9例(急性B淋巴细胞白血病1例、急性T淋巴细胞白血病8例),急性髓系白血病3例。所有患者确诊后接受诱导化疗,17例患者化疗后获得完全缓解,后续所有患者接受异基因造血干细胞移植。移植前状态:第1次完全缓解15例,第2次完全缓解1例,部分缓解1例,未缓解1例。所有患者完全植入,中性粒细胞植活和血小板植活的中位时间分别为12、13 d。随访时间4~80个月,中位数23个月,18例患者中15例存活,死亡3例,死因均为移植后复发。行allo-HSCT后,5例复发,移植后预期3年总生存(OS)率为83.3%±15.2%,无复发生存(RFS)率为55.4%±20.7%。15例移植前获得完全缓解的患者中,单倍体移植和同胞全相合移植的OS及RFS差异无统计学意义(均P>0.05)。结论:allo-HSCT可改善SET-NUP214+AL预后,使患者获得长期生存,复发是影响生存的重要因素。Objective To investigate the clinical efficacy of allogeneic hematopoietic stem cell transplantation(allo-HSCT)for patients with acute leukemia who are positive for the SET-NUP214 fusion gene(SET-NUP214+AL).Methods This was a retrospective case series study.Clinical data of 18 patients with SET-NUP214+AL who received allo-HSCT in the First Affiliated Hospital of Soochow University and Soochow Hongci Hematology Hospital from December 2014 to October 2021 were retrospectively analyzed to investigate treatment efficacy and prognosis.The Kaplan-Meier method was used for survival analysis.Results Of the 18 patients,12 were male and 6 were female,and the median age was 29 years(range,13-55 years).There were six cases of mixed phenotype acute leukemia(three cases of myeloid/T,two cases of B/T,one case of myeloid/B/T),nine cases of acute lymphoblastic leukemia(ALL)(one case of B-ALL and eight cases of T-ALL),and three cases of acute myeloid leukemia.All patients received induction chemotherapy after diagnosis,and 17 patients achieved complete remission(CR)after chemotherapy.All patients subsequently received allo-HSCT.Pre-transplantation status:15 patients were in the first CR,1 patient was in the second CR,1 was in partial remission,and 1 patient did not reach CR.All patients were successfully implanted with stem cells.The median time of granulocyte and platelet reconstitution was+12 and+13 days,respectively.With a median follow-up of 23(4-80)months,15 patients survived,while 3 patients died.The cause of death was recurrence of SET-NUP214+AL after transplantation.After allo-HSCT,5 patients relapsed.The estimated 3-year overall survival(OS)and relapse-free survival(RFS)rates were 83.3%±15.2%and 55.4%±20.7%,respectively.Among the 15 patients who achieved CR before transplantation,there was no significant difference in OS and RFS between haploidentical HSCT and matched sibling donor HSCT(all P>0.05).Conclusions Allo-HSCT can improve the prognosis and long-term survival rate of patients with SET-NUP214+AL.Disease recurren

关 键 词：白血病 造血干细胞移植 预后 白血病 急性 融合基因 SET-NUP214 异基因造血干细胞移植 

分 类 号：R733.71[医药卫生—肿瘤]