Myelodysplastic and myeloproliferative neoplasms: updates on the overlap syndromes

Leuk Lymphoma. 2018 Apr;59(4):803-812. doi: 10.1080/10428194.2017.1357179. Epub 2017 Aug 3.

Abstract

Myelodysplastic and myeloproliferative neoplasms (MDS/MPN) is a rare and distinct group of myeloid neoplasms with overlapping MDS and MPN features. Next generation sequencing studies have led to an improved understanding of MDS/MPN disease biology by identifying recurrent somatic mutations. Combining the molecular findings to patho-morphologic features has improved the precision of diagnosis and prognostic models in MDS/MPN. We discuss and highlight these updates in MDS/MPN nomenclature and diagnostic criteria per revised 2016 WHO classification of myeloid neoplasms in this article. There is an ongoing effort for data integration allowing for comprehensive genomic characterization, development of improved prognostic tools, and investigation for novel therapies using an international front specific for MDS/MPN. In this article, we discuss updates in prognostic models and current state of treatment for MDS/MPN.

Keywords: 2016 WHO myeloid classification; CMML; MDS/MPN-RS-T; atypical CML.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents, Immunological / therapeutic use*
  • Disease-Free Survival
  • High-Throughput Nucleotide Sequencing
  • Humans
  • Incidence
  • Leukocyte Count
  • Models, Biological*
  • Mutation
  • Myelodysplastic-Myeloproliferative Diseases / classification
  • Myelodysplastic-Myeloproliferative Diseases / drug therapy
  • Myelodysplastic-Myeloproliferative Diseases / genetics*
  • Myelodysplastic-Myeloproliferative Diseases / mortality*
  • Neoplasm Recurrence, Local / epidemiology*
  • Prognosis
  • Syndrome
  • Treatment Outcome

Substances

  • Antineoplastic Agents, Immunological