Canterbury DHB

Context

Long-term Outcomes in PV and Management of Transformation

The course of PV may reflect both its natural history and the effects of any treatment given.

Overall, median survival is approximately 13–14 years, and although many patients do very well, life expectancy is reduced compared to age-matched controls. There is an increased risk of:

Outcomes for patients who develop fibrosis and, in particular, acute myeloid leukemia (AML) are poor. No treatment has been shown to alter the natural history of PV, apart from allogeneic transplantation.

Options for patients with disease progression include:

The significant risks of allogeneic transplant, median age of presentation and long natural history of the disease do not justify it early in PV or ET, but it may have a role in:

For diagnostic criteria for post-PV and post-ET fibrosis, see Verstovsek S (2016),9 table 3.

About this Canterbury DHB document (6276):

Document Owner:

Sean Macpherson (see Who's Who)

Issue Date:

August 2018

Next Review:

April 2021

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 6276