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Polycythemia Vera-Associated Complications: Pathogenesis, Clinical Manifestations, And Effects On Outcomes

Authors Cuthbert D, Stein BL

Received 18 June 2019

Accepted for publication 18 September 2019

Published 18 October 2019 Volume 2019:10 Pages 359—371

DOI https://doi.org/10.2147/JBM.S189922

Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 2

Editor who approved publication: Dr Martin Bluth


Danielle Cuthbert,1 Brady Lee Stein2

1McGaw Medical Center of Northwestern University, Department of Internal Medicine, Chicago, IL 60611, USA; 2Northwestern University Feinberg School of Medicine, Division of Hematology/Oncology, Department of Medicine, Chicago, IL 60611, USA

Correspondence: Brady Lee Stein
Northwestern University Feinberg School of Medicine, Division of Hematology/Oncology, Department of Medicine, 645 N. Michigan Avenue, Suite 1020, Chicago, IL 60611, USA
Tel +1 312 695 6832
Fax +1 312 695 7814
Email bstein@nm.org

Abstract: Polycythemia vera is a Philadelphia-negative chronic myeloproliferative neoplasm, characterized by erythrocytosis, which is unique, compared to essential thrombocytosis and primary myelofibrosis. Though longevity can usually be expected, vascular morbidity is associated with this condition, as well as a propensity to evolve into myelofibrosis (post-PV MF) and acute myeloid leukemia. In addition, patients can have a pronounced symptom burden. Herein, contributors to the symptomatic burden, as well as the thrombotic and transformative tendencies are reviewed. From a symptom perspective, some are explained by cytokine release, others by microvascular complications, whereas certain symptoms can herald disease evolution. Thrombosis has multifactorial contributors, including but not limited to gender, and inflammatory stress; investigators have recently hypothesized that microparticles and Neutrophil Extracellular Trap Formations may add to thrombotic burden. Finally, we examine the progression to post-PV MF as well as leukemic transformation, highlighting well-established risk factors including age and leukocytosis, certain treatments, and the presence of “non-driver” mutations.

Keywords: polycythemia vera, symptomatic burden, thrombosis, myelofibrosis, leukemic transformation


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