Recent advances in the genomics and therapy of BCR/ABL1-positive and -negative chronic myeloproliferative neoplasms

Leuk Res. 2018 Apr;67:67-74. doi: 10.1016/j.leukres.2018.02.008. Epub 2018 Feb 14.


This review is based on the presentations and deliberations at the 7th John Goldman Chronic Myeloid Leukemia (CML) and Myeloproliferative Neoplasms (MPN) Colloquium which took place in Estoril, Portugal on the 15th October 2017, and the 11th post-ASH International Workshop on CML and MPN which took place on the 6th-7th December 2016, immediately after the 58th American Society of Hematology Annual Meeting. Rather than present a resume of the proceedings, we have elected to address some of the topical translational research and clinically relevant topics in greater detail. We address recent updates in the genetics and epigenetics of MPN, the mechanisms of transformation by mutant calreticulin, advances in the biology and therapy of systemic mastocytosis, clinical updates on JAK2 inhibitors and other therapeutic approaches for patients with MPNs, cardiovascular toxicity related to tyrosine kinase inhibitors and the concept of treatment-free remission for patients with CML.

Keywords: CALR; CML; Cardiotoxicity; JAK2 inhibitors; MPN; Mastocytosis; TFR.

Publication types

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

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Calreticulin / genetics
  • Cardiovascular Diseases / chemically induced
  • Cell Transformation, Neoplastic
  • Chronic Disease
  • Congresses as Topic
  • Epigenesis, Genetic
  • Fusion Proteins, bcr-abl / genetics*
  • Humans
  • Janus Kinase 2 / antagonists & inhibitors
  • Mastocytosis, Systemic / drug therapy
  • Mutation
  • Myeloproliferative Disorders / classification
  • Myeloproliferative Disorders / diagnosis
  • Myeloproliferative Disorders / drug therapy*
  • Myeloproliferative Disorders / genetics*
  • Protein Kinase Inhibitors / adverse effects
  • Protein Kinase Inhibitors / therapeutic use*
  • Remission Induction
  • Staurosporine / analogs & derivatives
  • Staurosporine / therapeutic use
  • Translational Medical Research


  • Antineoplastic Agents
  • BCR-ABL1 fusion protein, human
  • CALR protein, human
  • Calreticulin
  • Protein Kinase Inhibitors
  • Fusion Proteins, bcr-abl
  • JAK2 protein, human
  • Janus Kinase 2
  • Staurosporine
  • midostaurin