Revision of childhood acute erythroid leukemia in the diagnosis by World Health Organization 2016 classification
10.3760/cma.j.cn101070-20201104-01721
- VernacularTitle:世界卫生组织2016分型标准对儿童急性红白血病再评价
- Author:
Xia CHEN
1
;
Min RUAN
;
Ye GUO
;
Wenyu YANG
;
Fang LIU
;
Yuanyuan REN
;
Xiaoyan ZHANG
;
Beibei ZHAO
;
Xiaofan ZHU
Author Information
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
- Keywords:
Leukemia;
Acute erythroid leukemia;
Child
- From:
Chinese Journal of Applied Clinical Pediatrics
2022;37(5):338-342
- CountryChina
- Language:Chinese
-
Abstract:
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 CD 117, CD 13, CD 33, CD 34, CD7, 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.
