世界卫生组织2016分型标准对儿童急性红白血病再评价  

Revision of childhood acute erythroid leukemia in the diagnosis by World Health Organization 2016 classification

作　　者：陈霞 阮敏 郭晔 杨文钰 刘芳 任媛媛 张小燕 赵贝贝 竺晓凡 Chen Xia;Ruan Min;Guo Ye;Yang Wenyu;Liu Fang;Ren Yuanyuan;Zhang Xiaoyan;Zhao Beibei;Zhu Xiaofan(Pediatric Blood Disease Center,State Key Laboratory of Experimental Hematology,National Clinical Research Center for Blood Disease,Institute of Hematology&Blood Disease Hospital,Chinese Academy of Medical Sciences&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),北京协和医学院,实验血液学国家重点实验室,国家血液系统疾病临床医学研究中心,儿童血液病诊疗中心,天津300020

出　　处：《中华实用儿科临床杂志》2022年第5期338-342,共5页Chinese Journal of Applied Clinical Pediatrics

基　　金：中央高校基本科研业务费-青年教师(3332021058)。

摘　　要：目的应用世界卫生组织(WHO)2016分型标准再次评价经FAB形态学分型诊断的儿童急性红白血病(AEL)资料,了解该病的临床特征和预后。方法回顾性分析2002年1月1日至2019年12月31日就诊于中国医学科学院血液病医院儿童血液病诊疗中心的初诊白血病患儿,首先根据FAB分型诊断标准,明确诊断AEL,然后按WHO 2016分型标准进行再次评价。结果1.按FAB分型共20例患儿诊断为AEL,依据WHO 2016分型标准,其中11例诊断为骨髓增生异常综合征(MDS)伴原始细胞增多,3例诊断为急性髓系白血病(AML)伴MDS相关改变,1例诊断为急性单核细胞白血病,5例诊断为纯红白血病(PEL)。2.AEL骨髓形态学易见病态造血,原始细胞偶见Auer小体。髓系抗原主要表达CD117、CD_(13)、CD33、CD_(34)、CD_(7)、CD_(38)。染色体核型分析显示6例有异常染色体核型,涉及+8,-7,22p+,t(3;5:?),+3q-,15q-,del(9)(q13);12例为正常核型。3.13例进行化疗,1个疗程缓解率为38.5%,截至2020年7月1日,仅2例患儿无病生存(总生存分别为49个月及11个月)。结论儿童AEL易见三系病态造血,早期治疗反应差,预后欠佳,根据WHO 2016分型标准再评价后,大部分修订诊断为MDS相关。WHO 2016分型标准更贴近疾病本质,据此调整治疗可能有利于改善预后。Objective To analyze the relationship between FAB morphological classification and World Health Organization(WHO)2016 classification in children with acute erythroid leukemia(AEL),and to summarize the clinical features and prognosis.Methods Clinical data of de nova childhood AEL patients from January 1,2002 to December 31,2019,in Pediatric Blood Disease Center,Institute of Hematology&Blood Disease Hospital were retrospectively analyzed.All of them were re-evaluated according to the WHO 2016 classification.Results(1)A total of 20 patients were diagnosed as AEL by FAB classification.According to the criteria of WHO 2016,they were re-diagnosed as myelodysplastic syndromes(MDS)-refractory anemia with excess of blasts(11 cases),acute myeloid leukemia with MDS-related changes(3 cases),acute monocytic leukemia(1 case),and pure red leukemia(PEL,5 cases).(2)Pathological hematopoiesis was frequently detected in bone marrow smears.Auer bodies were seen occasionally in some blasts.The most common antigen expressing were CD117,CD_(13),CD33,CD_(34),CD_(7),and CD_(38).Karyotype analysis was performed in 18 cases successfully,involving 6 cases with abnormal karyotypes,including+8,-7,22p+,t(3;5:?),+3q-,15q-,and del(9)(q13).(3)Thirteen cases were treated by chemotherapy,and the one-course complete remission rate was 38.5%.By July 1,2020,only 2 cases were alive without disease.The overall survival was 49 months and 11 months,respectively.Conclusions Childhood AEL is susceptible to pathological hematopoiesis,poor response to early chemotherapy and poor prognosis.After re-evaluation according to WHO 2016 classification,most of them were diagnosed as MDS-related.Therefore,adjusting the suitable induction regimen with allogeneic hematopoietic stem cell transplantation may improve the prognosis.

关 键 词：白血病 急性红白血病 儿童 

分 类 号：R733.71[医药卫生—肿瘤]