Canterbury DHB

Context

Long-term Outcomes in ET and Management of Transformation

The course of ET may reflect both its natural history and the effects of any treatment given. Many patients have near-normal life expectancy, but there is an increased risk of:

Retrospective studies suggest incidence:

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 ET apart from allogeneic transplantation, which is not indicated for stable disease.

Options for patients with disease progression include:

The significant risks of allogeneic transplantation, median age of presentation, and long natural history of the disease do not justify it in early ET. However, it may have a role in:

See the WHO criteria for diagnosing post-ET myelofibrosis, box 23.2.

About this Canterbury DHB document (536518):

Document Owner:

Andrew Butler (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: 536518