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Pathology
Neoplastic clonal disorder of bone marrow stem cells causing excessive production of erythrocytes (often with neutrophils and platelets too)
Aetiology
95% is caused by mutation of JAK-2
Signs
Plethoric complexion, hepatosplenomegaly, thrombosis, hypertension
Symptoms
Often asymptomatic, headache, drowsiness, symptoms of thrombosis, pruritus, haemorrhage, pruritus in water
Investigations
Bloods: FBC shows increased Hb, White Cells and Platelets, Clotting, U&E, JAK-2
mutation analysis.
If JAK 2 is positive, no further investigation required
If JAK 2 negative: serum erythropoietin, bone marrow aspirate
Treatment
Conservative: Reduce risk factors for thrombosis
Medical: Venesection, low dose chemotherapy, Aspirin, Folic acid
Prognosis
Median survival approximately 14 years if treated
Transformation of PV to myelofibrosis or Acute Myeloid Leukaemia
Key Facts
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Key References
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