21例Ph阳性急性淋巴细胞白血病儿童不同治疗疗效分析  

Analysis of the Different Therapeutic Effects in 21 Ph^+Acute Lymphoblastic Leukemia Children

作　　者：王书春 郭晔 陈晓娟 刘晓明 阮敏 杨文钰 张丽 邹尧 陈玉梅 竺晓凡 WANG Shu-Chun;GUO Ye;CHEN Xiao-Juan;LIU Xiao-Ming;RUAN Min;YANG Wen-Yu;ZHANG Li;ZOU Yao;CHEN Yu-Mei;ZHU Xiao-Fan(State Key Laboratory of Experimental Hematology,National Clinical Research Center for Blood Disease,Institute of Hematology&Blood Diseases Hospital,Chinese Academy of Medical Science&Peking Union Medical College,Tianjin 300020,China)

机构地区：[1]中国医学科学院血液病医院(中国医学科学院血液学研究所),实验血液学国家重点实验室,国家血液病临床医学研究中心,天津300020

出　　处：《中国实验血液学杂志》2021年第1期38-42,共5页Journal of Experimental Hematology

摘　　要：目的:分析Ph^+急性淋巴细胞白血病(ALL)患儿TKI联合化疗和异基因造血干细胞移植(allo-HSCT)的疗效。方法:回顾性分析2008年1月至2015年4月在中国医学科学院血液病医院儿童血液病中心就诊应用CCLG-2008-ALL方案治疗的21例年龄<12岁Ph^+ALL患儿,将患者分为2组:化疗联合TKI治疗组和allo-HSCT组,分析患者的转归情况。结果:21例患者中,男性17例,女性4例,中位年龄为8(4-12)岁,中位随访时间为58(10-133)个月。所有患儿均采用CCLG-2008-ALL高危组治疗方案诱导化疗,14例酪氨酸激酶抑制剂联合高危组巩固化疗的患者,至末次随访时间,9例患者获得长期生存;治疗3个月时,对于未达到分子生物学缓解(5例)、复发重新获得缓解(2例)有强烈移植愿望(2例)的患者行异基因造血干细胞移植,9例移植患者中6例患者获得长期生存。结论:在TKI时代,Ph^+ALL患儿通过TKI联合强化疗可以获得与allo-HSCT相当的5年无疾病进展生存率。对于不能获得分子生物学缓解或者复发的患者,allo-HSCT仍然有着不可撼动的地位。Objective:To analyze the outcomes of the children suffered from Philadelphia chromosome positive acute lymphoblastic leukemia(Ph^+ALL)treated with tyrosine kinase inhibitor(TKI)plus chemotherapy and allogeneic hematopoietic stem cell transplantation(allo-HSCT).Methods:21 cases of firstly diagnosed Ph^+ALL patients aged<12 year treated with Chinese Childhood Leukemia Group ALL 2008(CCLG-ALL 2008)protocol form January 2008 and April 2015 were retrospectively analyzed.The patients were divided into two groups,one group was TKI+chemotherapy group,the other group was allo-HSCT group.Results:Among 21 patients,17 were male and 4 were female with a median age of 8 years old(range,4-12 years),the median follow-up time was 30 moths(range,10-133 months).All the patients were treated with chemotherapy induced by the high-risk project of CCLG-ALL 2008.Among 14 patients treated with TKI plus chemotherapy,nine patients achieved complete remission.During 3 months after treatment,patients without complete molecular response or with the second complete remission and intensity desire of transplantation were treated with allo-HSCT,among 9 patients with allo-HSCT,six patients achieved long term survival.Conclusion:At TKI era,TKI combined with strong chemotherapy can make Ph^+ALL children achieve 5 years event-free survival as campared those treated with allo-HSCT.However,for the patients without complete molecular response persistently and relapsed they can still benefit from allo-HSCT.

关 键 词：急性淋巴细胞白血病 酪氨酸激酶抑制剂 造血干细胞移植 PH阳性 儿童 

分 类 号：R733.71[医药卫生—肿瘤]