Canterbury DHB

Context

Polycythaemia Vera (PV)

Polycythaemia vera is a chronic MPN characterised by excess RBC production independent of the normal regulatory mechanisms. In virtually all patients, this is due to a mutation in the JAK2 gene, usually V617F but occasionally a functionally similar mutation in exon 12.

Generally, two phases of PV are recognised:

Natural progression of PV also includes a low incidence of evolution to myelodysplastic/pre-leukaemic phase, and/or a blast phase (i.e. acute leukaemia).

The term PV should not be used to describe an isolated increased red cell mass, which is more accurately described as primary or secondary erythrocytosis.

In This Section

Diagnostic Criteria

Clinical Assessment for Raised Hb

Investigation of Suspected PV

Treatment

Response Assessment

Referral to GP for Shared Care

PV Refractory or Intolerant Hydroxyurea

Long-term Outcomes in PV and Management of Transformation

Further Reading

About this Canterbury DHB document (6225):

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: 6225