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01-12-2014 | Review Article

Polycythemia vera disease burden: contributing factors, impact on quality of life, and emerging treatment options

Authors: Brady L. Stein, Alison R. Moliterno, Ramón V. Tiu

Published in: Annals of Hematology | Issue 12/2014

Abstract

Polycythemia vera (PV) is a chronic myeloproliferative neoplasm characterized by clonal expansion of a hematopoietic progenitor, erythrocytosis, often leukocytosis and/or thrombocytosis, and nearly always an activating mutation in Janus kinase 2 (JAK2). The PV symptom burden can be considerable, in part driven by small or large vessel thrombotic tendency, splenomegaly, fatigue, pruritus, and a chronic risk of disease transformation to myelofibrosis or acute myeloid leukemia. In addition, patients with PV have an increased risk of mortality compared with the general population that often results from cardiovascular complications or disease transformation. Further, healthcare utilization and costs are higher in patients with PV than noncancer controls. First-line therapy options for high-risk patients may effectively manage PV in some instances; however, some patients do not receive adequate benefit from current treatment options and experience a more severe disease burden as a result. This may be especially true for those patients who are resistant to or intolerant of hydroxyurea or interferon-based therapies. New treatments currently being investigated in phase 3 clinical trials may alleviate disease burden in this patient population.

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Title: Polycythemia vera disease burden: contributing factors, impact on quality of life, and emerging treatment options
Authors: Brady L. Stein
Alison R. Moliterno
Ramón V. Tiu
Publication date: 01-12-2014
Publisher: Springer Berlin Heidelberg
Published in: Annals of Hematology / Issue 12/2014
Print ISSN: 0939-5555
Electronic ISSN: 1432-0584
DOI: https://doi.org/10.1007/s00277-014-2205-y

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