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

Journal Article (Journal Article;Review)

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.

Full Text

Duke Authors

Cited Authors

  • Stein, BL; Gotlib, J; Arcasoy, M; Nguyen, MH; Shah, N; Moliterno, A; Jamieson, C; Pollyea, DA; Scott, B; Wadleigh, M; Levine, R; Komrokji, R; Klisovic, R; Gundabolu, K; Kropf, P; Wetzler, M; Oh, ST; Ribeiro, R; Paschal, R; Mohan, S; Podoltsev, N; Prchal, J; Talpaz, M; Snyder, D; Verstovsek, S; Mesa, RA

Published Date

  • April 2015

Published In

Volume / Issue

  • 13 / 4

Start / End Page

  • 424 - 434

PubMed ID

  • 25870379

Pubmed Central ID

  • PMC8161684

Electronic International Standard Serial Number (EISSN)

  • 1540-1413

Digital Object Identifier (DOI)

  • 10.6004/jnccn.2015.0058


  • eng

Conference Location

  • United States