Polycythaemia Vera
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  • 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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Dr Tom Leach

Dr Tom Leach MBChB DCH EMCert(ACEM) currently works as a GP Registrar and an Emergency Department CMO in Australia. He is also a Clinical Associate Lecturer at the Australian National University. After graduating from his medical degree at the University of Manchester in 2011, Tom completed his Foundation Training at Bolton Royal Hospital, before moving to Australia in 2013. He started almostadoctor whilst a third year medical student in 2009. Read full bio

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