Polycythaemia vera is a myeloproliferative disorder that results in an excess of red blood cells in the bloodstream.
The estimated prevalence is around 2-3 per 10000 people. It typically presents in older individuals. There may be a slightly greater male predilection.
JAK2 mutations (particularly the V617F mutation) can be found in more than 95% of people with polycythaemia vera.
Radiographic manifestations are varied and and non-specific can affect a number of system and can include:
- organ infarcts such as splenic infarcts
- features of extramedullary haematopoiesis
- increased radiographic attenuation of cerebral arteries, veins and sinuses (may mimic cerebral vein thrombosis)
- splenic infarction
- venous thromboses
- cerebrovascular events
- myocardial infarction
Treatment and prognosis
The overall prognosis can be variable with a wide natural course amongst individuals. Treatment options include venesection, myelosuppressive medications, interferon, aspirin, Anagrelide hydrochloride (Agrylin®) and radioactive phosphorus (P-32).
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