Canterbury DHB
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.
Topic Code: 6225