title: Current and future treatment options for polycythemia vera.
abstract: Patients with polycythemia vera (PV), a myeloproliferative neoplasm characterized by an elevated red blood cell mass, are at high risk of vascular and thrombotic complications and have reduced quality of life due to a substantial symptom burden that includes pruritus, fatigue, constitutional symptoms, microvascular disturbances, and bleeding. Conventional therapeutic options aim at reducing vascular and thrombotic risk, with low-dose aspirin and phlebotomy as first-line recommendations for patients at low risk of thrombotic events and cytoreductive therapy (usually hydroxyurea or interferon alpha) recommended for high-risk patients. However, long-term effective and well-tolerated treatments are still lacking. The discovery of mutations in Janus kinase 2 (JAK2) as the underlying molecular basis of PV has led to the development of several targeted therapies, including JAK inhibitors, and results from the first phase 3 clinical trial with a JAK inhibitor in PV are now available. Here, we review the current treatment landscape in PV, as well as therapies currently in development.
authors.affiliation: Johannes Wesling Academic Medical Center, Minden, Germany, martin.griesshammer@muehlenkreiskliniken.de.
authors.forename: Martin
authors.initials: M
authors.lastname: Griesshammer
authors.forename: Heinz
authors.initials: H
authors.lastname: Gisslinger
authors.forename: Ruben
authors.initials: R
authors.lastname: Mesa
mesh_terms: D000818:Animals; D001241:Aspirin / Q000627:therapeutic use; D005544:Forecasting; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D053614:Janus Kinase 2 / Q000037:antagonists & inhibitors* / Q000378:metabolism; D018962:Phlebotomy / Q000639:trends; D011087:Polycythemia Vera / Q000175:diagnosis* / Q000201:enzymology / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D016898:Interferon-alpha; C507924:JAK2 protein, human; D053614:Janus Kinase 2; D001241:Aspirin
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