title: Evolving Therapeutic Options for Polycythemia Vera: Perspectives of the Canadian Myeloproliferative Neoplasms Group.
abstract: 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.
authors.affiliation: Division of Hematology, Jewish General Hospital, Montreal, Quebec, Canada.
authors.forename: Shireen
authors.initials: S
authors.lastname: Sirhan
authors.affiliation: Hematopoiesis and Aging Research Unit, Hôpital Maisonneuve-Rosemont, Montreal, Quebec, Canada.
authors.forename: Lambert
authors.initials: L
authors.lastname: Busque
authors.affiliation: Division of Hematology, St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Lynda
authors.initials: L
authors.lastname: Foltz
authors.affiliation: Faculty of Medicine, Memorial University, St. John's, Newfoundland, Canada.
authors.forename: Kuljit
authors.initials: K
authors.lastname: Grewal
authors.affiliation: Western University, Department of Oncology, Windsor Site, Windsor, Ontario, Canada.
authors.forename: Caroline
authors.initials: C
authors.lastname: Hamm
authors.affiliation: Thunder Bay Regional Health Sciences, Department of Oncology, Lakehead University, Thunder Bay, Ontario, Canada.
authors.forename: Nicole
authors.initials: N
authors.lastname: Laferriere
authors.affiliation: Department of Oncology, McGill University, Montreal, Quebec, Canada.
authors.forename: Pierre
authors.initials: P
authors.lastname: Laneuville
authors.affiliation: Department of Medicine, Hematology and Thromboembolism, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Brian
authors.initials: B
authors.lastname: Leber
authors.affiliation: Division of Hematology, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Elena
authors.initials: E
authors.lastname: Liew
authors.affiliation: Department of Hematology and Transfusional Medicine, Centre Hospitalier de l'Université de Montréal, Montreal, Quebec, Canada.
authors.forename: Harold J
authors.initials: HJ
authors.lastname: Olney
authors.affiliation: Department of Oncology, McGill University, Montreal, Quebec, Canada.
authors.forename: Jaroslav
authors.initials: J
authors.lastname: Prchal
authors.affiliation: Department of Pathology, University Health Network, Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Anna
authors.initials: A
authors.lastname: Porwit
authors.affiliation: Medical Oncology and Hematology, Princess Margaret Cancer Centre, University of Toronto, Toronto, Ontario, Canada. Electronic address: vikas.gupta@uhn.ca.
authors.forename: Vikas
authors.initials: V
authors.lastname: Gupta
mesh_terms: D000368:Aged; D000963:Antimetabolites / Q000009:adverse effects / Q000627:therapeutic use*; D002170:Canada; D004359:Drug Therapy, Combination; D006801:Humans; D006918:Hydroxyurea / Q000009:adverse effects / Q000627:therapeutic use*; D008875:Middle Aged; D011087:Polycythemia Vera / Q000175:diagnosis / Q000188:drug therapy*; D011379:Prognosis; D011788:Quality of Life; D018570:Risk Assessment
chemical_list: D000963:Antimetabolites; D006918:Hydroxyurea
keywords: Hydroxyurea; Interferon; JAK2 mutation; Polycythemia vera; Ruxolitinib