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Revision of childhood acute erythroid leukemia in the diagnosis by World Health Organization 2016 classification / 中华实用儿科临床杂志
Chinese Journal of Applied Clinical Pediatrics ; (24): 338-342, 2022.
Article in Chinese | WPRIM | ID: wpr-930431
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.

Full text: Available Index: WPRIM (Western Pacific) Type of study: Diagnostic study / Prognostic study Language: Chinese Journal: Chinese Journal of Applied Clinical Pediatrics Year: 2022 Type: Article

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Full text: Available Index: WPRIM (Western Pacific) Type of study: Diagnostic study / Prognostic study Language: Chinese Journal: Chinese Journal of Applied Clinical Pediatrics Year: 2022 Type: Article