Evidence map›Paper›PMID 39182032›Full record

ArticleBMC pediatrics2024

NUP98::NSD1 and FLT3/ITD co-expression is an independent predictor of poor prognosis in pediatric AML patients.

Jing-Wen Wang, Yu-Li, Xing-Ge Yang, Lu-Hong Xu

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Article in BMC pediatrics, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers, 2 of them syntheses that pooled it.

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5citing papers in PubMed, 2 pooled it
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1 · What the graph read from it

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3 · Its place in the literature

Who cites it

5 citing papers in PubMed, 2 syntheses or guidelines pooled it.

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  4. Strategies for identifyingHaematologica · 2026
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4 · The record

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5 · Who and what money

Authors and funding

4 authors.

Jing-Wen WangDepartment of Pediatrics, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China.
Yu-LiDepartment of Pediatrics, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China.
Xing-Ge YangDepartment of Pediatrics, The First Affiliated Hospital of Henan University of Science and Technology, Luoyang, Henan, China. 13783186162@163.com.
Lu-Hong XuDepartment of Pediatrics, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China. xulhong@mail.sysu.edu.cn.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivePatients who carry NUP98::NSD1 or FLT3/ITD mutations are reported to have poor prognosis. Previous studies have confidently reported that the poor outcome in younger AML patients is owning to dual NUP98::NSD1 and FLT3/ITD positivity, with a high overlap for those two genetic lesions. In this study, we assessed the prognostic value of the presence of both NUP98::NSD1 and FLT3/ITD in pediatric AML patients.

methodsWe screened a large cohort of 885 pediatric cases from the COG-National Cancer Institute (NCI) TARGET AML cohort and found 57 AML patients with NUP98 rearrangements.

resultsThe frequency of NUP98 gene fusion was 10.8% in 529 patients. NUP98::NSD1 fusion was the most common NUP98 rearrangement, with a frequency of 59.6%(34 of 57). NUP98::NSD1 -positive patients who carried FLT3/ITD mutations had a decreased CR1 or CR2 rate than those patients carried FLT3/ITD mutation alone (P = 0.0001). Moreover, patients harboring both NUP98::NSD1 fusion and FLT3/ITD mutation exhibited inferior event-free survival (EFS, P < 0.001) and overall survival (OS, P = 0.004) than patients who were dual negative for these two genetic lesions. The presence of only NUP98::NSD1 fusion had no significant impact on EFS or OS. We also found that cases with high FLT3/ITD AR levels ( > = 0.5) with or without NUP98::NSD1 had inferior prognosis. Multivariate analysis demonstrated that the presence of both NUP98::NSD1 and FLT3/ITD was an independent prognostic factors for EFS (hazard ratio: 3.2, P = 0.001) in patients with pediatric AML. However, there was no obvious correlation with OS (hazard ratio: 1.3, P = 0.618). Stem cell transplantation did not improve the survival rate of cases with NUP98 fusion or NUP98::NSD1 AML in terms of EFS or OS.

conclusionPresence of both NUP98::NSD1 and FLT3/ITD was found to be an independent factor for dismal prognosis in pediatric AML patients. Notably, lack of FLT3/ITD mutations in NUP98::NSD1 -positive patients did not retain its prognostic value.

Indexed as

fms-Like Tyrosine Kinase 3Leukemia, Myeloid, AcuteMutationNuclear Pore Complex ProteinsAdolescentChildChild, PreschoolFemaleHistone-Lysine N-MethyltransferaseHumansInfantIntracellular Signaling Peptides and ProteinsMaleNuclear ProteinsOncogene Proteins, FusionPrognosisFLT3 protein, humanfms-Like Tyrosine Kinase 3Histone-Lysine N-MethyltransferaseIntracellular Signaling Peptides and ProteinsNuclear Pore Complex ProteinsNuclear ProteinsNUP98-NSD1 protein, humanOncogene Proteins, FusionAcute myeloid leukemia (AML)FLT3/ITDNucleoporin gene 98NUP98::NSD1Pediatric

Identifiers

PMID39182032
PMCPMC11344362

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.