Polycythaemia Vera
- Polycythaemia is a raised RBC count, haemoglobin and packed cell volume/haematocrit
- Absolute increase in red cell mass: polycythemia vera, secondary to hypoxia or abnormal EPO secretion
- Apparent polycythemia: plasma volume reduced, red cell mass unchanged
- If packed cell volume/haematocrit persistently raised (male >0.52, female >0.48) this warrants investigation
- Polycythemia vera myeloproliferative disorder, 90% have JAK2 (Janus kinase 2) mutation
- Presentation: headaches, dizziness, lethargy, sweating and pruritus
- Increased risk of thrombosis (especially strokes) and bleeding (hyperviscosity and platelet dysfunction)
- Increased cell turnover may lead to gout
- Signs: plethora, rosacea, palpable splenomegaly
- Investigations: elevated WCC, elevated platelets, iron deficiency, EPO estimation normal or low
- Bone marrow: hypercellularity
- Management: aspirin (75mg/day), venesection (haematocrit to <0.45)
- Advanced disease: hydroxycarbamide to suppress erythropoiesis
- Median survival >10 years, 10% transform to myelofibrosis, 5% to AML
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