Incorporating mutations and bone marrow fibrosis into the revised international prognostic scoring system in myelodysplastic syndromes

Leuk Lymphoma. 2024 Jan;65(1):100-108. doi: 10.1080/10428194.2023.2271593. Epub 2024 Jan 10.

Abstract

The independent prognostic significance of bone marrow fibrosis (BMF) in myelodysplastic syndromes (MDS) is challenged under currently molecular prognostic models. In this study, the clinical and genetic data from 438 MDS patients were analyzed retrospectively. The patients were randomly divided into training (n = 306) and validation (n = 132) cohorts. The independent significant prognostic factors included age, IPSS-R, BMF, TP53 and U2AF1. Using their weighted coefficients, we developed a simplified prognostic system. Four risk groups were produced: low, intermediate, high and very high. The new model yielded more clearly separated survival curves than the IPSS-R. In addition, our model achieved higher C-indexes (0.61 in the training cohort and 0.63 in the validation cohort) than the IPSS-RM model (0.59 and 0.58) and IPSS-R (0.57 and 0.56). In conclusion, BMF was an independent significant prognostic factor for MDS, and adding BMF into the IPSS-R improved its predictive capability.

Keywords: Myelodysplastic syndromes; TP53; bone marrow fibrosis; model; prognosis.

Publication types

  • Randomized Controlled Trial

MeSH terms

  • Humans
  • Mutation
  • Myelodysplastic Syndromes* / diagnosis
  • Myelodysplastic Syndromes* / genetics
  • Primary Myelofibrosis*
  • Prognosis
  • Retrospective Studies