儿童急性白血病短暂性自发缓解3例并文献复习  

Transient spontaneous remission of childhood acute leukemia:report of 3 cases and review of literature

作　　者：周林英 许吕宏[2] 刘勇[2] 王健[2] 王茵 周敦华[2] 方建培[2] Zhou Linying;Xu Lyuhong;Liu Yong;Wang Jian;Wang Yin;Zhou Dunhua;Fang Jianpei(Department of Pediatrics,the Second Affiliated Hospital of Guangxi Medical University,Nanning 530007,China;Department of Pediatrics,Sun Yat-sen Memorial Hospital,Sun Yat-sen University,Guangzhou 510120,China)

机构地区：[1]广西医科大学第二附属医院儿科,南宁530007 [2]中山大学孙逸仙纪念医院儿科,广州510120

出　　处：《白血病．淋巴瘤》2022年第5期294-297,共4页Journal of Leukemia & Lymphoma

摘　　要：目的探讨儿童急性白血病短暂性自发缓解的临床特征及可能原因。方法收集分别在2018年7月、2019年5月、2020年10月于中山大学孙逸仙纪念医院就诊的在规范化疗前出现短暂性自发缓解的3例急性白血病患儿资料。复习相关文献,探讨白血病自发缓解的相关影响因素。结果3例患儿起病时均有发热症状,经抗感染治疗后获得短暂性自发缓解。例1在初次诊断急性B淋巴细胞白血病(B-ALL)后部分缓解,白血病基因检查示E2A-PBX1阳性,12 d后复发;例2在初次诊断B-ALL后自行缓解,白血病基因检查示p16基因缺失、NRAS和EP300突变阳性,20 d后复发;例3在初次诊断急性单核细胞白血病后自行缓解,白血病基因检查示MLL-ENL阳性、NRAS突变阳性,30 d后复发。复习文献显示,白血病自发缓解的主要影响因素有唐氏综合征、感染和输血,其他影响因素还有白血病相关基因、终止妊娠、药物的应用等。结论儿童急性白血病短暂性自发缓解在临床中少见,可能的机制与感染导致免疫异常有关。对于白血病自发缓解状态的患者需密切监测微小残留病。Objective To investigate the clinical characteristics and possible causes of transient spontaneous remission of childhood acute leukemia.Methods The data of 3 children with acute leukemia who had transient spontaneous remission before standardized chemotherapy in Sun Yat-sen Memorial Hospital of Sun Yat-sen University in July 2018,May 2019 and October 2020 were collected.Moreover,the related influencing factors of spontaneous remission in leukemia were discussed by review of the literature.Results All 3 children had fever at the onset of the disease,and they achieved transient spontaneous remission after anti-infection therapy.Case 1 obtained partial remission after the initial diagnosis of acute B lymphocytic leukemia(B-ALL),leukemia gene test showed E2A-PBX1 fusion,and relapsed after 12 days.Case 2 obtained spontaneous remission after the initial diagnosis of B-ALL,leukemia gene test showed p16 gene deletion and NRAS and EP300 genes mutation,and relapsed after 20 days.Case 3 obtained spontaneous remission after the initial diagnosis of acute monocytic leukemia,leukemia gene test showed MLL-ENL fusion and NRAS gene mutation,and relapsed after 30 days.A review of the literature showed that the main influencing factors of spontaneous remission in leukemia were Down syndrome,infection and blood transfusion.Other influencing factors included leukemia-related genes,termination of pregnancy and application of drugs.Conclusions Transient spontaneous remission of childhood acute leukemia is rare in clinical practice,and the possible mechanism is related to infection-induced immune abnormalities.It is recommended that leukemia patients with spontaneous remission should be closely monitored for minimal residual disease.

关 键 词：白血病 儿童 缓解 自行 复发 

分 类 号：R733.71[医药卫生—肿瘤]