More than a century has elapsed since the appearance of the modern descriptions of polycythemia vera (PV). During this time, much has been learned regarding disease pathogenesis and PV-associated molecular aberrations. New information has allowed amendments to traditional diagnostic criteria. Phlebotomy remains the cornerstone treatment of PV, whereas myelosuppressive agents may augment the benefit of using phlebotomy for thrombosis prevention in high-risk patients. Excessive aspirin use is contraindicated in PV, although the use of lower-dose aspirin has been shown to be safe and effective in alleviating microvascular symptoms including erythromelalgia and headaches. Recent studies have shown the utility of selective serotonin receptor antagonists for treating PV-associated pruritus. Nevertheless, many questions remain unanswered. What is the specific genetic mutation or altered molecular pathway that is causally related to the disease? In the absence of a specific molecular marker, how is a working diagnosis of PV made? What evidence supports current practice in the management of PV? This article summarizes both old and new information on PV; proposes a modern diagnostic algorithm to formulate a working diagnosis; and provides recommendations for patient management, relying whenever possible on an evidence-based approach.
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