Novel insights into the biology and treatment of chronic myeloproliferative neoplasms

Tariq I. Mughal, Tiziano Barbui, Omar Abdel-Wahab, Robert Kralovics, Catriona Jamieson, Hans Michael Kvasnicka, Ann Mullaly, Raajit Rampal, Ruben Mesa, Jean Jacques Kiladjian, Michael Deininger, Josef Prchal, Rüdiger Hehlmann, Giuseppe Saglio, Richard A. Van Etten

Research output: Contribution to journalReview article

3 Scopus citations

Abstract

Myeloproliferative neoplasms (MPNs) are clonal disorders of hematopoiesis characterized by a high frequency of genetic alterations, and include chronic myeloid leukemia (CML) and the BCR-ABL1-negative MPNs. Herein we summarize recent advances and controversies in our understanding of the biology and therapy of these disorders, as discussed at the 8th post-American Society of Hematology CML-MPN workshop. The principal areas addressed include the breakthrough discovery of CALR mutations in patients with JAK2/MPL wild type MPN, candidate therapies based on novel genetic findings in leukemic transformation and new therapeutic targets in MPNs, and an appraisal of bone marrow histopathology in MPNs with a focus on the potential new clinical entity of "masked" polycythemia vera. An update on clinical trials of Janus kinase (JAK) inhibitors is presented as well as current understanding regarding the definitions and mechanisms of resistance to JAK inhibitors, and updated information on the safety and efficacy of discontinuation of tyrosine kinase inhibitors in patients with CML.

Original languageEnglish (US)
Pages (from-to)1938-1948
Number of pages11
JournalLeukemia and Lymphoma
Volume56
Issue number7
DOIs
StatePublished - Jul 1 2015

Keywords

  • Molecular genetics
  • Myeloid leukemias and dysplasias
  • Myeloproliferative disorders
  • Prognostication
  • Signaling therapies

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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  • Cite this

    Mughal, T. I., Barbui, T., Abdel-Wahab, O., Kralovics, R., Jamieson, C., Kvasnicka, H. M., Mullaly, A., Rampal, R., Mesa, R., Kiladjian, J. J., Deininger, M., Prchal, J., Hehlmann, R., Saglio, G., & Van Etten, R. A. (2015). Novel insights into the biology and treatment of chronic myeloproliferative neoplasms. Leukemia and Lymphoma, 56(7), 1938-1948. https://doi.org/10.3109/10428194.2014.974594