The Challenge of Treating Myelodysplastic Syndromes/Myeloproliferative Neoplasms

Clin Lymphoma Myeloma Leuk. 2017 Jul:17S:S37-S42. doi: 10.1016/j.clml.2017.02.012.

Abstract

Myelodysplastic syndromes/myeloproliferative neoplasms (MDS/MPN) comprise a spectrum of myeloid disorders with both dysplastic and proliferative features, arising from hematopoietic stem cells. MDS/MPN are neither MDS nor MPN but a distinct World Health Organization-defined subclassification of diseases that, by definition, do not arise from a pre-existing MDS or MPN, inherently creating diagnostic and therapeutic challenges. Appropriately recognizing and classifying MDS/MPN is paramount for appropriately formulating treatment strategies and accurate prognostication. Highlighting the unique molecular, morphologic, and clinical characteristics among the subclassifications of MDS/MPN can assist in the appropriate diagnosis and aid in choice of therapeutic strategies. We describe the challenges of the appropriate diagnosis and treatment of MDS/MPN.

Keywords: CMML; MDS/MPN; MDS/MPN-U; Overlap syndromes; aCML.

Publication types

  • Review

MeSH terms

  • Adult
  • Azacitidine / therapeutic use*
  • DNA (Cytosine-5-)-Methyltransferases / antagonists & inhibitors
  • DNA (Cytosine-5-)-Methyltransferases / metabolism
  • Enzyme Inhibitors / therapeutic use
  • Humans
  • Myelodysplastic Syndromes / classification
  • Myelodysplastic Syndromes / diagnosis
  • Myelodysplastic Syndromes / drug therapy*
  • Myeloproliferative Disorders / classification
  • Myeloproliferative Disorders / diagnosis
  • Myeloproliferative Disorders / drug therapy*
  • Survival Analysis
  • World Health Organization

Substances

  • Enzyme Inhibitors
  • DNA (Cytosine-5-)-Methyltransferases
  • Azacitidine