Historical views, conventional approaches, and evolving management strategies for myeloproliferative neoplasms

J Natl Compr Canc Netw. 2015 Apr;13(4):424-34. doi: 10.6004/jnccn.2015.0058.


The classical Philadelphia chromosome-negative myeloproliferative neoplasms (MPN), which include essential thrombocythemia, polycythemia vera, and myelofibrosis (MF), are in a new era of molecular diagnosis, ushered in by the identification of the JAK2(V617F) and cMPL mutations in 2005 and 2006, respectively, and the CALR mutations in 2013. Coupled with increased knowledge of disease pathogenesis and refined diagnostic criteria and prognostic scoring systems, a more nuanced appreciation has emerged of the burden of MPN in the United States, including the prevalence, symptom burden, and impact on quality of life. Biological advances in MPN have translated into the rapid development of novel therapeutics, culminating in the approval of the first treatment for MF, the JAK1/JAK2 inhibitor ruxolitinib. However, certain practical aspects of care, such as those regarding diagnosis, prevention of vascular events, choice of cytoreductive agent, and planning for therapies, present challenges for hematologists/oncologists, and are discussed in this article.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Calreticulin / genetics
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Hydroxyurea / therapeutic use
  • Janus Kinase 2 / genetics
  • Janus Kinases / antagonists & inhibitors
  • Polycythemia Vera / diagnosis
  • Polycythemia Vera / genetics*
  • Polycythemia Vera / therapy*
  • Primary Myelofibrosis / diagnosis
  • Primary Myelofibrosis / genetics*
  • Primary Myelofibrosis / therapy*
  • Protein Kinase Inhibitors / therapeutic use
  • Receptors, Thrombopoietin / genetics
  • Splenomegaly / etiology
  • Splenomegaly / therapy
  • Thrombocythemia, Essential / diagnosis
  • Thrombocythemia, Essential / genetics*
  • Thrombocythemia, Essential / therapy*
  • Thrombosis / etiology
  • Thrombosis / prevention & control


  • Antineoplastic Agents
  • Calreticulin
  • Protein Kinase Inhibitors
  • Receptors, Thrombopoietin
  • MPL protein, human
  • JAK2 protein, human
  • Janus Kinase 2
  • Janus Kinases
  • Hydroxyurea