Myeloproliferative Neoplasms, Version 2.2017, NCCN Clinical Practice Guidelines in Oncology

J Natl Compr Canc Netw. 2016 Dec;14(12):1572-1611. doi: 10.6004/jnccn.2016.0169.

Abstract

Myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET) are a group of heterogeneous disorders of the hematopoietic system collectively known as Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs). The diagnosis and the management of patients with MPNs have evolved since the identification of mutations that activate the JAK pathway (JAK2, CALR, and MPL mutations) and the development of targeted therapies has resulted in significant improvements in disease-related symptoms and quality of life. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnostic workup of MPN (MF, PV, and ET), risk stratification, treatment, and supportive care strategies for the management of MF.

Publication types

  • Practice Guideline

MeSH terms

  • Calreticulin / genetics
  • Calreticulin / metabolism
  • Humans
  • Janus Kinase 2 / genetics
  • Janus Kinase 2 / metabolism
  • Medical Oncology / standards*
  • Mutation
  • Philadelphia Chromosome
  • Polycythemia Vera* / diagnosis
  • Polycythemia Vera* / epidemiology
  • Polycythemia Vera* / genetics
  • Polycythemia Vera* / therapy
  • Prevalence
  • Primary Myelofibrosis* / diagnosis
  • Primary Myelofibrosis* / epidemiology
  • Primary Myelofibrosis* / genetics
  • Primary Myelofibrosis* / therapy
  • Quality of Life
  • Receptors, Thrombopoietin / genetics
  • Receptors, Thrombopoietin / metabolism
  • Risk Assessment
  • Signal Transduction
  • Thrombocythemia, Essential* / diagnosis
  • Thrombocythemia, Essential* / epidemiology
  • Thrombocythemia, Essential* / genetics
  • Thrombocythemia, Essential* / therapy

Substances

  • CALR protein, human
  • Calreticulin
  • Receptors, Thrombopoietin
  • MPL protein, human
  • JAK2 protein, human
  • Janus Kinase 2