Chronic myeloproliferative disorder, persistent increase in platelet count

  • Associated with thrombotic or haemorrhagic complications, average age 60 years
  • Good prognosis, risk of transformation to myelofibrosis and AML
  • Symptoms: may be asymptomatic, burning on the soles and palms, cold peripheries, headache, dizziness
  • Occlusion of arterioles: ischaemia, gangrene or acrocyanosis.
  • Haemorrhagic complications: ecchymosis, epistaxis, menorrhagia and GIT haemorrhage.
  • Splenomegaly unusual, may be painful splenic infarction
  • Platelet count usually >600×109/l
  • Diagnosis of exclusion (rule out infection, inflammation, iron deficiency, malignancy)
  • Investigations: normal ESR, plasma viscosity and fibribnogen levels
  • 50% have acquired JAK2 mutation.
  • Bone marrow examination: exclude CML, myelofibrosis and myelodysplasia, check iron stores
  • Treatment: hydroxycarbamide and aspirin is given in high risk cases (elderly, platelets >1500×109/l or previous thromboembolic events)
  • Low-dose asprin alone in lower risk patients, interferon given in pregnancy

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