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Peer-reviewedCC BYZhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi

[Clinical analysis of nine pediatric cases of acute myeloid leukemia with NUP98 rearrangement].

  1. 1Department of Pediatric Hematology and Oncology, Children's Hospital of First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.

Abstract

A retrospective case analysis was conducted on nine newly diagnosed pediatric patients with NUP98-rearranged acute myeloid leukemia (AML) who were admitted to the First Affiliated Hospital of Zhengzhou University between January 2019 to June 2025. Nine patients (3 males and 6 females) accounted for 4.2% of the 214 children diagnosed with AML during the same period. The patients' median age at diagnosis was 7 years (range: 8 months to 14 years). This type of AML was classified as M₁, M₄, and M₅, with M₅ being the most common subtype (7/9, 77.8%). The most common fusion gene type was NUP98::NSD1 (6/9, 66.7%), which was frequently accompanied by gene mutations such as FLT3-ITD and NRAS. Six cases had normal chromosome karyotypes, whereas the remaining 3 cases had abnormal karyotypes. The complete remission (CR) rate after one course of induction chemotherapy was 77.8% (7/9), and four of the children with CR underwent bridging allogeneic hematopoietic stem cell transplantation (allo-HSCT) after two to three courses of consolidation therapy, and all survived without disease. The remaining three children who continued chemotherapy had a relapse and died; whereas the two children who did not achieve CR died due to disease progression. In conclusion, at this center, pediatric AML with NUP98 rearrangement was more common in females and was often seen in the M₅ subtype. NUP98::NSD1 was the most common fusion gene and was often accompanied by gene mutations, such as FLT3-ITD. Although the induction remission rate was high, the disease was prone to relaps. Allo-HSCT may improve the prognosis of these children.

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BibTeX
@article{wang2026clinical,
  title = {{[Clinical analysis of nine pediatric cases of acute myeloid leukemia with NUP98 rearrangement].}},
  author = {C M Wang and X P Jia and J Luo and Y S Zhang and X J Xu and B Li and D Wang and Q H Yao and G Y Sheng},
  journal = {Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi},
  year = {2026},
  month = aug,
  doi = {10.3760/cma.j.cn121090-20260319-00142},
  url = {https://europepmc.org/article/MED/42851181},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - [Clinical analysis of nine pediatric cases of acute myeloid leukemia with NUP98 rearrangement].
AU  - C M Wang
AU  - X P Jia
AU  - J Luo
AU  - Y S Zhang
AU  - X J Xu
AU  - B Li
AU  - D Wang
AU  - Q H Yao
AU  - G Y Sheng
PY  - 2026
DA  - 2026/08/01
JO  - Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
DO  - 10.3760/cma.j.cn121090-20260319-00142
UR  - https://europepmc.org/article/MED/42851181
AB  - A retrospective case analysis was conducted on nine newly diagnosed pediatric patients with NUP98-rearranged acute myeloid leukemia (AML) who were admitted to the First Affiliated Hospital of Zhengzhou University between January 2019 to June 2025. Nine patients (3 males and 6 females) accounted for 4.2% of the 214 children diagnosed with AML during the same period. The patients' median age at diagnosis was 7 years (range: 8 months to 14 years). This type of AML was classified as M₁, M₄, and M₅, with M₅ being the most common subtype (7/9, 77.8%). The most common fusion gene type was NUP98::NSD1 (6/9, 66.7%), which was frequently accompanied by gene mutations such as FLT3-ITD and NRAS. Six cases had normal chromosome karyotypes, whereas the remaining 3 cases had abnormal karyotypes. The complete remission (CR) rate after one course of induction chemotherapy was 77.8% (7/9), and four of the children with CR underwent bridging allogeneic hematopoietic stem cell transplantation (allo-HSCT) after two to three courses of consolidation therapy, and all survived without disease. The remaining three children who continued chemotherapy had a relapse and died; whereas the two children who did not achieve CR died due to disease progression. In conclusion, at this center, pediatric AML with NUP98 rearrangement was more common in females and was often seen in the M₅ subtype. NUP98::NSD1 was the most common fusion gene and was often accompanied by gene mutations, such as FLT3-ITD. Although the induction remission rate was high, the disease was prone to relaps. Allo-HSCT may improve the prognosis of these children.
SN  - 0253-2727
SN  - 2707-9740
ER  -