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:
- presence of 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).
- 1. Hirsch IS. Pulmonary Changes in Polycythemia Vera. Radiology 1936 26:4, 469-473 Available at: http://pubs.rsna.org/doi/abs/10.1148/26.4.469.
- 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