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Pediatric non–Down’s syndrome acute megakaryoblastic leukemia patients in China: A single center's real-world analysis

Non-Down’s syndrome acute megakaryocytic leukemia (non-DS-AMKL) is a subtype of childhood acute myeloid leukemia (AML), whose prognosis, prognostic factors and treatment recommendations have not yet to be defined in children. We conducted a retrospective study with 65 newly diagnosed non-DS-AMKL chi...

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Autores principales: Zhang, Aoli, Liu, Lipeng, Zong, Suyu, Chen, Xiaoyan, Liu, Chao, Chang, Lixian, Chen, Xiaojuan, Yang, Wenyu, Guo, Ye, Zhang, Li, Zou, Yao, Chen, Yumei, Zhang, Yingchi, Ruan, Min, Zhu, Xiaofan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9577933/
https://www.ncbi.nlm.nih.gov/pubmed/36267971
http://dx.doi.org/10.3389/fonc.2022.940725
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author Zhang, Aoli
Liu, Lipeng
Zong, Suyu
Chen, Xiaoyan
Liu, Chao
Chang, Lixian
Chen, Xiaojuan
Yang, Wenyu
Guo, Ye
Zhang, Li
Zou, Yao
Chen, Yumei
Zhang, Yingchi
Ruan, Min
Zhu, Xiaofan
author_facet Zhang, Aoli
Liu, Lipeng
Zong, Suyu
Chen, Xiaoyan
Liu, Chao
Chang, Lixian
Chen, Xiaojuan
Yang, Wenyu
Guo, Ye
Zhang, Li
Zou, Yao
Chen, Yumei
Zhang, Yingchi
Ruan, Min
Zhu, Xiaofan
author_sort Zhang, Aoli
collection PubMed
description Non-Down’s syndrome acute megakaryocytic leukemia (non-DS-AMKL) is a subtype of childhood acute myeloid leukemia (AML), whose prognosis, prognostic factors and treatment recommendations have not yet to be defined in children. We conducted a retrospective study with 65 newly diagnosed non-DS-AMKL children from August 2003 to June 2020 to investigate the clinical impact of factors and clinical outcome. Among all 65 patients, 47 of them were treated at our center who received three different regimens due to time point of admission (CAMS-another, CAMS-2009 and CAMS-2016 protocol), and the efficacy were compared. Patients with newly diagnosed non-DS-AMKL accounted for 7.4% of pediatric AML cases. The median age of the patients was 18 months at diagnosis, and over 90% of them were under three-years-old. The overall survival (OS) rates were 33.3% ± 1.7%, 66.7% ± 24.4% and 74.2% ± 4.0% for three groups (CAMS-another, CAMS-2009 and CAMS-2016 regimen), respectively. In CAMS-2016 group, the complete remission (CR) rate after induction was 67.7% (21/31), while the total CR rate after all phases of chemotherapy was 80.6% (25/31). The 2-year survival probability did not significantly improve in patients underwent HSCT when compared with non-HSCT group (75.0% ± 4.7% vs. 73.9% ± 4.6%, p=0.680). Those who had a “dry tap” during BM aspiration at admission had significantly worse OS than those without “dry tap” (33.3% ± 8.6% vs. 84.0% ± 3.6%, p=0.006). Moreover, the results also revealed that patients with CD34+ had significantly lower OS (50.0% ± 6.7% vs. 89.5% ± 3.5%, p=0.021), whereas patients with CD36+ had significantly higher OS than those who were negative (85.0% ± 4.0% vs. 54.5% ± 6.6%, p=0.048). In conclusion, intensive chemotherapy resulted in improved prognosis of non-DS-AMKL children and subclassification may base on “dry tap” and immunophenotypic. Although some progress has been made, outcomes of non-DS-AMKL children remain unsatisfactory, especially in HSCT group, when compared with other AML types.
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spelling pubmed-95779332022-10-19 Pediatric non–Down’s syndrome acute megakaryoblastic leukemia patients in China: A single center's real-world analysis Zhang, Aoli Liu, Lipeng Zong, Suyu Chen, Xiaoyan Liu, Chao Chang, Lixian Chen, Xiaojuan Yang, Wenyu Guo, Ye Zhang, Li Zou, Yao Chen, Yumei Zhang, Yingchi Ruan, Min Zhu, Xiaofan Front Oncol Oncology Non-Down’s syndrome acute megakaryocytic leukemia (non-DS-AMKL) is a subtype of childhood acute myeloid leukemia (AML), whose prognosis, prognostic factors and treatment recommendations have not yet to be defined in children. We conducted a retrospective study with 65 newly diagnosed non-DS-AMKL children from August 2003 to June 2020 to investigate the clinical impact of factors and clinical outcome. Among all 65 patients, 47 of them were treated at our center who received three different regimens due to time point of admission (CAMS-another, CAMS-2009 and CAMS-2016 protocol), and the efficacy were compared. Patients with newly diagnosed non-DS-AMKL accounted for 7.4% of pediatric AML cases. The median age of the patients was 18 months at diagnosis, and over 90% of them were under three-years-old. The overall survival (OS) rates were 33.3% ± 1.7%, 66.7% ± 24.4% and 74.2% ± 4.0% for three groups (CAMS-another, CAMS-2009 and CAMS-2016 regimen), respectively. In CAMS-2016 group, the complete remission (CR) rate after induction was 67.7% (21/31), while the total CR rate after all phases of chemotherapy was 80.6% (25/31). The 2-year survival probability did not significantly improve in patients underwent HSCT when compared with non-HSCT group (75.0% ± 4.7% vs. 73.9% ± 4.6%, p=0.680). Those who had a “dry tap” during BM aspiration at admission had significantly worse OS than those without “dry tap” (33.3% ± 8.6% vs. 84.0% ± 3.6%, p=0.006). Moreover, the results also revealed that patients with CD34+ had significantly lower OS (50.0% ± 6.7% vs. 89.5% ± 3.5%, p=0.021), whereas patients with CD36+ had significantly higher OS than those who were negative (85.0% ± 4.0% vs. 54.5% ± 6.6%, p=0.048). In conclusion, intensive chemotherapy resulted in improved prognosis of non-DS-AMKL children and subclassification may base on “dry tap” and immunophenotypic. Although some progress has been made, outcomes of non-DS-AMKL children remain unsatisfactory, especially in HSCT group, when compared with other AML types. Frontiers Media S.A. 2022-10-04 /pmc/articles/PMC9577933/ /pubmed/36267971 http://dx.doi.org/10.3389/fonc.2022.940725 Text en Copyright © 2022 Zhang, Liu, Zong, Chen, Liu, Chang, Chen, Yang, Guo, Zhang, Zou, Chen, Zhang, Ruan and Zhu https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Zhang, Aoli
Liu, Lipeng
Zong, Suyu
Chen, Xiaoyan
Liu, Chao
Chang, Lixian
Chen, Xiaojuan
Yang, Wenyu
Guo, Ye
Zhang, Li
Zou, Yao
Chen, Yumei
Zhang, Yingchi
Ruan, Min
Zhu, Xiaofan
Pediatric non–Down’s syndrome acute megakaryoblastic leukemia patients in China: A single center's real-world analysis
title Pediatric non–Down’s syndrome acute megakaryoblastic leukemia patients in China: A single center's real-world analysis
title_full Pediatric non–Down’s syndrome acute megakaryoblastic leukemia patients in China: A single center's real-world analysis
title_fullStr Pediatric non–Down’s syndrome acute megakaryoblastic leukemia patients in China: A single center's real-world analysis
title_full_unstemmed Pediatric non–Down’s syndrome acute megakaryoblastic leukemia patients in China: A single center's real-world analysis
title_short Pediatric non–Down’s syndrome acute megakaryoblastic leukemia patients in China: A single center's real-world analysis
title_sort pediatric non–down’s syndrome acute megakaryoblastic leukemia patients in china: a single center's real-world analysis
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9577933/
https://www.ncbi.nlm.nih.gov/pubmed/36267971
http://dx.doi.org/10.3389/fonc.2022.940725
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