Evolving Therapeutic Options for Polycythemia Vera : Perspectives of the Canadian Myeloproliferative Neoplasms Group
(2015) In Clinical Lymphoma, Myeloma and Leukemia 15(12). p.715-727- 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... (More)
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.
(Less)
- author
- publishing date
- 2015-12-01
- type
- Contribution to journal
- publication status
- published
- keywords
- Hydroxyurea, Interferon, JAK2 mutation, Polycythemia vera, Ruxolitinib
- in
- Clinical Lymphoma, Myeloma and Leukemia
- volume
- 15
- issue
- 12
- pages
- 715 - 727
- publisher
- CIG Media Group
- external identifiers
-
- pmid:26433906
- scopus:84952631295
- ISSN
- 2152-2650
- DOI
- 10.1016/j.clml.2015.07.650
- language
- English
- LU publication?
- no
- id
- b81c3512-e809-4f72-a34f-4c6b539cc865
- date added to LUP
- 2019-05-22 09:40:43
- date last changed
- 2024-01-01 06:37:03
@misc{b81c3512-e809-4f72-a34f-4c6b539cc865,
abstract = {{<p>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.</p>}},
author = {{Sirhan, Shireen and Busque, Lambert and Foltz, Lynda and Grewal, Kuljit and Hamm, Caroline and Laferriere, Nicole and Laneuville, Pierre and Leber, Brian and Liew, Elena and Olney, Harold J. and Prchal, Jaroslav and Porwit, Anna and Gupta, Vikas}},
issn = {{2152-2650}},
keywords = {{Hydroxyurea; Interferon; JAK2 mutation; Polycythemia vera; Ruxolitinib}},
language = {{eng}},
month = {{12}},
number = {{12}},
pages = {{715--727}},
publisher = {{CIG Media Group}},
series = {{Clinical Lymphoma, Myeloma and Leukemia}},
title = {{Evolving Therapeutic Options for Polycythemia Vera : Perspectives of the Canadian Myeloproliferative Neoplasms Group}},
url = {{http://dx.doi.org/10.1016/j.clml.2015.07.650}},
doi = {{10.1016/j.clml.2015.07.650}},
volume = {{15}},
year = {{2015}},
}