Novel insights into the biology and treatment of chronic myeloproliferative neoplasms Journal Article

   


Authors: 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.
Article Title: Novel insights into the biology and treatment of chronic myeloproliferative neoplasms
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. © 2014 Informa UK, Ltd.
Keywords: myeloproliferative disorders; janus kinase inhibitor; histopathology; review; molecular genetics; bone marrow; chronic myeloid leukemia; wild type; janus kinase; cancer stem cell; myeloproliferative neoplasm; polycythemia vera; myeloid leukemias and dysplasias; myeloid leukemia; prognostication; clonal evolution; indel mutation; rna editing; long term survival; human; priority journal; signaling therapies
Journal Title: Leukemia and Lymphoma
Volume: 56
Issue: 7
ISSN: 1042-8194
Publisher: Taylor & Francis Group  
Date Published: 2015-01-01
Start Page: 1938
End Page: 1948
Language: English
DOI: 10.3109/10428194.2014.974594
PROVIDER: scopus
PUBMED: 25330439
PMCID: PMC5076856
DOI/URL:

http://www.scopus.com/inward/record.url?eid=2-s2.0-84938149109&partnerID=40&md5=83cc84cdb407a2b7ed7105f20b8a1561
Notes: Export Date: 2 September 2015 -- Source: Scopus
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MSK Authors
  1. Raajit Kumar Rampal
    338 Rampal
  2. Omar Ibrahim Abdel-Wahab
    573 Abdel-Wahab
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