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Very important developments related to polycythemia vera (PV) have occurred during the last two decades. The discovery  of Janus kinase (JAK) 2 mutations has changed both the diagnosis and clinical management of PV. Currently JAK2 molecular testing  is essential in the diagnostic work-up and JAK2 mutation positivity is a major diagnostic criterion. The discovery of JAK2 mutations  suggested  that  abnormal  JAK-STAT  signaling  was  a  pivotal  feature  in  the  pathogenesis  of  Philadelphia-negative  myeloproliferative   neoplasms. This idea led to the development of JAK inhibitors. Currently ruxolitinib, a JAK1/JAK2 inhibitor, is also approved for PV  patients with hydroxyurea resistance or intolerance. International collaborations have made it possible to describe disease characteristics  and evolution better. Presently it is possible to quantify the symptomatic burden of the disease and to estimate prognosis. In spite of  these  developments,  management  of  PV  still  largely  depends  on  estimation  of  thromboembolic  risk  and  trying  to  decrease  the  risk   with or without cytoreductive medications. Different approaches have been proposed by international disease experts for the diagnosis,  thromboembolic  risk  estimation,  and  drug  selection.  This  paper  aims  to  review  clinical  aspects  of  PV  and  propose  a  management   algorithm. The authors also point to still unresolved questions and unmet needs in diagnosis and management.

Polycythemia vera: diagnosis, clinical course, and current management