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Clin Lymphoma Myeloma Leuk. 2015 Dec;15(12):715-27. doi: 10.1016/j.clml.2015.07.650. Epub 2015 Aug 7.

Evolving Therapeutic Options for Polycythemia Vera: Perspectives of the Canadian Myeloproliferative Neoplasms Group.

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Division of Hematology, Jewish General Hospital, Montreal, Quebec, Canada.
Hematopoiesis and Aging Research Unit, Hôpital Maisonneuve-Rosemont, Montreal, Quebec, Canada.
Division of Hematology, St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
Faculty of Medicine, Memorial University, St. John's, Newfoundland, Canada.
Western University, Department of Oncology, Windsor Site, Windsor, Ontario, Canada.
Thunder Bay Regional Health Sciences, Department of Oncology, Lakehead University, Thunder Bay, Ontario, Canada.
Department of Oncology, McGill University, Montreal, Quebec, Canada.
Department of Medicine, Hematology and Thromboembolism, McMaster University, Hamilton, Ontario, Canada.
Division of Hematology, University of Alberta, Edmonton, Alberta, Canada.
Department of Hematology and Transfusional Medicine, Centre Hospitalier de l'Université de Montréal, Montreal, Quebec, Canada.
Department of Pathology, University Health Network, Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.
Medical Oncology and Hematology, Princess Margaret Cancer Centre, University of Toronto, Toronto, Ontario, Canada. Electronic address:


Polycythemia vera (PV) is a clonal stem cell disorder characterized by erythrocytosis and associated with burdensome symptoms, reduced quality of life, risk of thrombohemorrhagic complications, and risk of transformation to myelofibrosis and acute myeloid leukemia. The discovery of the JAK2 V617 mutation marked a significant milestone in understanding the pathophysiology of the disease and subsequently the diagnostic and therapeutic approaches. The current diagnostic criteria for PV are based on hemoglobin level and presence of the JAK2 V617 mutation. The treatment is geared toward prevention of thrombotic events, normalization of blood counts, control of disease-related symptoms, and potential prolongation of survival. Cytoreductive therapy is indicated in patients at increased risk of thrombosis. Hydroxyurea (HU) remains the most commonly used first-line cytoreductive therapy and is superior to phlebotomy in reducing risk of arterial and venous thrombosis. Interferon (IFN) is used either at failure of HU or in selected patients as first-line therapy. The results of pegylated IFN in phase 2 studies appear encouraging, with molecular responses occurring in some patients. Ongoing phase 3 studies of HU versus pegylated IFN will define the optimal first-line cytoreductive therapy for PV. A recent phase 3 trial has shown the superiority of the JAK1/2 inhibitor ruxolitinib in comparison to best available treatment in HU-intolerant or -resistant patients. The therapeutic landscape of PV is likely to change in the near future. In this report, we assess the potential impact of the changing landscape of PV management on daily practice.


Hydroxyurea; Interferon; JAK2 mutation; Polycythemia vera; Ruxolitinib

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