ABSTRACT
Introduction: Myeloproliferative neoplasms (MPNs) are associated with a variety of symptoms and signs which cause major morbidity for the patients. The disorders are associated with increased incidence of thromboembolic and hemorrhagic events which can lead to complications and shortened life expectancy.
Areas covered: Using systematic literature review and expert clinical and research experience the authors discuss strategies for the management of symptoms and signs including pruritus, fatigue, splenomegaly, and cytopenia. Cytoreduction including treatments to inhibit the JAK/STAT pathway are considered. Pathogenesis and prevention and treatment of thrombotic and hemorrhagic events and their management is addressed and the suggested management of the special situations such as surgery and pregnancy are discussed.
Expert commentary: Management of disease has traditionally focused on symptom treatment and complication prevention but the discovery of driver mutations has led to treatments aiming to eliminate the clone, which should be the ultimate goal of therapy. A future challenge is to develop safe and effective MPN therapy and to personalize therapy.
Declaration of interest
MF McMullin has been in the speakers’ bureau and served on advisory boards for Novartis and has served on an advisory board for Gilead. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. One peer reviewer on this manuscript declares consulting for Incyte Corporation for a non-interventional study regarding the natural history of polycythemia vera but has no other relevant financial relationships to disclose.