Myelodysplastic Syndrome Updated

Pathobiology. 2019;86(1):7-13. doi: 10.1159/000489702. Epub 2018 Jul 24.

Abstract

This review highlights the main changes in the revised 2016 WHO Classification of Myeloid Neoplasms (published in 2017) that impact the diagnosis and management of patients with myelodysplastic syndrome (MDS). The revision was based on data accumulated since the 2008 WHO classification of MDS, much of which relates to new molecular genetic information about these neoplasms. The new information has led to some reorganization of the MDS disease categories, including a broadening of the subset of cases classified as MDS with ring sideroblasts, many of which have mutations in the spliceosome gene SF3B1. Other revisions have refined the definitions of some disease categories to improve disease risk stratification. The revised categories in the new classification ensure that MDS patients receive risk-adapted therapies based on the most recently available data.

Keywords: Cytogenetics; Myelodysplastic syndrome; Myeloid neoplasms; Ring sideroblasts.

Publication types

  • Review

MeSH terms

  • Algorithms
  • Cytogenetics
  • Humans
  • Mutation
  • Myelodysplastic Syndromes / classification*
  • Myelodysplastic Syndromes / diagnosis
  • Myeloproliferative Disorders / classification*
  • Myeloproliferative Disorders / diagnosis
  • Phosphoproteins / genetics*
  • RNA Splicing Factors / genetics*
  • World Health Organization

Substances

  • Phosphoproteins
  • RNA Splicing Factors
  • SF3B1 protein, human