The estimated prevalence is around 2-3 per 10,000 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 non-specific and can affect a number of system, including:
- organ infarcts e.g. splenic infarcts
- features of extramedullary haematopoiesis
- increased radiographic attenuation of cerebral arteries, veins and dural 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, and aspirin.
Anagrelide is used when secondary thrombocythaemia is an issue. Oral radiophosphorus (32-P) is now avoided in 'benign' myeloproliferative diseases due to the risk of inducing acute myeloid leukaemia 5.
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- 2. Delamore IW, Macdonald AF, Samuel E. The radiological investigation of polycythaemia with particular emphasis on the renal tract. The British journal of radiology. 35: 671-8. doi:10.1259/0007-1285-35-418-671 - Pubmed
- 3. Healy JF, Nichols C. Polycythemia mimicking venous sinus thrombosis. AJNR. American journal of neuroradiology. 23 (8): 1402-3. Pubmed
- 4. Pitman RG, Steiner RE, Szur L. The radiological appearances of the chest in polycythaemia vera. Clinical radiology. 12: 276-85. Pubmed
- 5. Brandt L, Anderson H. Survival and risk of leukaemia in polycythaemia vera and essential thrombocythaemia treated with oral radiophosphorus: are safer drugs available?. (1995) European journal of haematology. 54 (1): 21-6. Pubmed