Polycythaemia vera is a myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets. It has a peak incidence in the sixth decade, with typical features including hyperviscosity, pruritus and splenomegaly.

Management

• aspirin
  • reduces the risk of thrombotic events
• venesection
  • first-line treatment to keep the haemoglobin in the normal range
• chemotherapy
  • hydroxyurea - slight increased risk of secondary leukaemia
  • phosphorus-32 therapy

Prognosis

• thrombotic events are a significant cause of morbidity and mortality
• 5-15% of patients progress to myelofibrosis
• 5-15% of patients progress to acute leukaemia (risk increased with chemotherapy treatment)

Ruxolitinib is a Janus kinase (JAK) 1 and 2 inhibitor which is used in the management of polycythaemia vera for prevention of thrombus formation. It is not a first-line management, and is used in patients who are resistant or intolerant to hydroxyurea.