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Pathology
Persistent thrombocytosis (>450 x 109/l) which is not reactive or due to another myeloproliferative neoplasm or myelodysplasia
Aetiology
Unknown, 50% have JAK-2 mutation, not associated with radiation, drugs, chemicals
Signs
Erythromelalgia (burning sensation and erythema in hands and feet caused by microthrombi), splenomegaly
Symptoms
Often asymptomatic, thrombosis, headache, light-headedness, haemorrhage
Investigations
Bloods: FBC shows platelets persistently >450 otherwise normal.
JAK-2 mutation is present in 50% of cases
Blood film: giant platelets and platelet clumps
Treatment
Conservative: Lifestyle changes to reduce clot risk, treat thrombotic risks
Medical: Busulfan, hydroxyurea or interferon-alpha
Prognosis
Life expectancy near normal
Risk of transformation to myelofibrosis or AML (less likely than in PV)
Key Facts
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