Wilms' tumor 1 expression combined with genetic mutations for prognostic assessment in MDS

Leuk Lymphoma. 2023 Apr;64(4):856-864. doi: 10.1080/10428194.2023.2185086. Epub 2023 Mar 11.

Abstract

Overexpression of Wilms' tumor (WT1) is frequently observed in myelodysplastic syndrome (MDS), which has been proposed as a prognostic marker. However, the prognostic role of WT1 expression in different contexts remains to be fully elucidated. We retrospectively assessed the relationships between WT1 levels and preexisting prognostic factors to further investigate its prognostic role under different contexts. In our study, WT1 expression was positively correlated with WHO 2016 classification and IPSS-R stratification. Lower WT1 expression was found in relation to TET2, TP53, CD101, or SRSF2 mutations, while mutant NPM1 patients possessed higher level. Notably, WT1 overexpression maintained its inferior prognostic effect on overall survival (OS) in TP53-wild patients but not in TP53-mutated group. In multivariate analysis, higher WT1 expression was a risk factors for OS in EB patients without TP53 mutations. Overall, WT1 expression was useful to predict prognosis for MDS and its prognostic role was impacted by some gene mutations.

Trial registration: ClinicalTrials.gov NCT02850822.

Keywords: Myelodysplastic syndromes; Wilms’ tumor 1; gene mutations; prognosis biomarker.

MeSH terms

  • Humans
  • Kidney Neoplasms*
  • Mutation
  • Myelodysplastic Syndromes* / diagnosis
  • Myelodysplastic Syndromes* / genetics
  • Prognosis
  • Retrospective Studies
  • WT1 Proteins* / genetics
  • WT1 Proteins* / metabolism
  • Wilms Tumor*

Substances

  • WT1 Proteins

Associated data

  • ClinicalTrials.gov/NCT02850822