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Review
. 2021 Apr;35(2):159-176.
doi: 10.1016/j.hoc.2020.12.001. Epub 2021 Jan 26.

Overview of Myeloproliferative Neoplasms: History, Pathogenesis, Diagnostic Criteria, and Complications

Douglas Tremblay  1 , Abdulraheem Yacoub  2 , Ronald Hoffman  3
Affiliations
Review

Overview of Myeloproliferative Neoplasms: History, Pathogenesis, Diagnostic Criteria, and Complications

Douglas Tremblay et al. Hematol Oncol Clin North Am. 2021 Apr.

Abstract

Myeloproliferative disorders are a group of diseases morphologically linked by terminal myeloid cell expansion that frequently evolve from one clinical phenotype to another and eventually progress to acute myeloid leukemia. Diagnostic criteria for the Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) have been established by the World Health Organization and they are recognized as blood cancers. MPNs have a complex and incompletely understood pathogenesis that includes systemic inflammation, clonal hematopoiesis, and constitutive activation of the JAK-STAT pathway. Complications, such as thrombosis and progression to overt forms of myelofibrosis and acute leukemia, contribute significantly to morbidity and mortality of patients with MPN.

Keywords: Diagnostic criteria; Essential thrombocythemia; History; Myelofibrosis; Myeloproliferative neoplasms; Polycythemia vera.

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Conflict of interest statement

Disclosure Dr R. Hoffman is a consultant for Protagonist and serves on Data Safety Monitoring Boards for Novartis and AbbVie. He also receives research support from Novartis, Dompe, Kartos, AbbVie, Scholar Rock, and Turning Point Therapeutics. Dr A. Yacoub is a consultant for Incyte and Novartis. He has stocks and ownership in Hylapharm.

Figures

Figure 1.
Figure 1.
Major clinical investigators responsible for early diagnostic and therapeutic advances in myeloproliferative neoplasms. Louis Henri Vaquez was the first to describe polycythemia vera, which was later expanded by William Osler. William Dameshek described pathologic links and coined the term “myeloproliferative disorders.” Philip Fialkow identified myeloproliferative neoplasms as a stem cell disease and Louis Wasserman formed the Polycythemia Vera Study Group, the first dedicated organization to study therapies in myeloproliferative neoplasms.
See this image and copyright information in PMC

References

    1. Dickstein JI, Vardiman JW. Hematopathologic findings in the myeloproliferative disorders. Semin Oncol 1995;22(4):355–373. - PubMed
    1. Barosi G, Mesa RA, Thiele J, et al. Proposed criteria for the diagnosis of post-polycythemia vera and post-essential thrombocythemia myelofibrosis: a consensus statement from the International Working Group for Myelofibrosis Research and Treatment. Leukemia 2008;22(2):437–438. - PubMed
    1. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016;127(20):2391–2405. - PubMed
    1. Rowley JD. Letter: A new consistent chromosomal abnormality in chronic myelogenous leukaemia identified by quinacrine fluorescence and Giemsa staining. Nature 1973;243(5405):290–293. - PubMed
    1. Cortes JE, Talpaz M, Giles F, et al. Prognostic significance of cytogenetic clonal evolution in patients with chronic myelogenous leukemia on imatinib mesylate therapy. Blood 2003;101(10):3794–3800. - PubMed

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