Polycythaemia vera

Keywords: PRV, PV, Erythrocytosis

Presentation

Appropriate Tests

A haemopoietic stem cell proliferative disorder with polycythaemia and variable associated neutrophilia and thrombocytosis. It is one of the Myeloproliferative neoplasms.

In ~5% of cases a transformation to acute leukaemia may occur. Bleeding and thrombosis commonly occur in this disorder. The primary management of PV is venesection, commonly to a Haematocrit of <45%. Many patients eventually develop myelofibrosis.

See Polycythaemia.

 

Erythrocytosis

 

Splenomegaly

 

Thrombosis (arterial or venous)

 

Hyperviscosity

 

Hyperuricaemia

 

Peptic ulcer

 

Pruritis

 

Hypertension

 

Bleeding

Often from local lesion (eg, peptic ulcer, epistaxis) but may be associated with platelet dysfunction.

See Bleeding disorders

Transformation to

 

  • Acute leukaemia

 

  • Myelodysplasia

See Myelodysplastic syndromes