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Review
. 2015 Nov 20;33(33):3953-60.
doi: 10.1200/JCO.2015.61.6474. Epub 2015 Aug 31.

Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F

Brady L Stein  1 , Stephen T Oh  1 , Dmitriy Berenzon  1 , Gabriela S Hobbs  1 , Marina Kremyanskaya  1 , Raajit K Rampal  1 , Camille N Abboud  1 , Kenneth Adler  1 , Mark L Heaney  1 , Elias J Jabbour  1 , Rami S Komrokji  1 , Alison R Moliterno  1 , Ellen K Ritchie  1 , Lawrence Rice  1 , John Mascarenhas  1 , Ronald Hoffman  2
Affiliations
Review

Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F

Brady L Stein et al. J Clin Oncol. .

Abstract

Polycythemia vera (PV) is a chronic myeloproliferative neoplasm that is associated with a substantial symptom burden, thrombohemorrhagic complications, and impaired survival. A decade after the seminal discovery of an activating mutation in the tyrosine kinase JAK2 in nearly all patients with PV, new treatment options are finally beginning to emerge, necessitating a critical reappraisal of the underlying pathogenesis and therapeutic modalities available for PV. Herein, we comprehensively review clinical aspects of PV including diagnostic considerations, natural history, and risk factors for thrombosis. We summarize recent studies delineating the genetic basis of PV, including their implications for evolution to myelofibrosis and secondary acute myeloid leukemia. We assess the quality of evidence to support the use of currently available therapies, including aspirin, phlebotomy, hydroxyurea, and interferon. We analyze recent studies evaluating the safety and efficacy of JAK inhibitors, such as ruxolitinib, and evaluate their role in the context of other available therapies for PV. This review provides a framework for practicing hematologists and oncologists to make rational treatment decisions for patients with PV.

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Conflict of interest statement

Authors' disclosures of potential conflicts of interest are found in the article online at www.jco.org. Author contributions are found at the end of this article.

Figures

Fig 1.
Fig 1.
Polycythemia vera (PV): the JAK2 discovery era, 2005 to present. ET, essential thrombocythemia; IFN, interferon; MF, myelofibrosis; MPN, myeloproliferative neoplasm; peg-IFN, pegylated IFN.
Fig 2.
Fig 2.
Patient-reported symptoms in polycythemia vera. Incidence of each symptom is estimated based on data from Scherber et al, Emmanuel et al, Johansson et al, and Abelsson et al.
See this image and copyright information in PMC

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