Polycythemia vera (PV) is a rare disease of the blood-forming cells in the bone marrow which in the first place leads to a permanent (chronic) increase in the numbers of red blood cells (erythrocytes). Thereby, the fluidity of the blood may be reduced, and circulatory disorders such as thrombosis and embolism may be the consequence. Most patients with polycythemia vera have a nearly normal life expectancy if they are treated adequately.
The cause of polycythemia vera is still unknown. It is almost always an acquired disease, but can in rare cases also be inborn. An increased production of red blood cells (erythrocytes) is characteristic for the disease.
At the onset of disease, symptoms are more likely to be unspecific.
A striking redness of the face may even give the impression that the person involved is particularly healthy.
Once the number of red blood cells reaches a critical value, the fluidity (viscosity) of the blood is decreased and circulatory disorders arise. This leads to a bluish colour of the skin (cyanosis).
Other symptoms may include: itching on the whole body (especially when taking a shower or bath – aquagenic pruritus), high blood pressure, headache, dizziness, painful circulatory disorders affecting the fingers and toes, tinnitus, nosebleed, and vision disorders.
The most frequent complications result from the increased viscosity of the blood which may lead to blood clots (thrombosis) or blockage of blood vessels (embolism), both in large and small vessels.
Such circulatory disorders can lead to the following diseases:
- thrombosis of the portal vein (impairing blood supply to the liver)
- thrombosis of a leg vein
- heart attack
- temporary interruption of blood flow to the brain (transient ischemic attack, TIA)
- pulmonary embolism.
The numbers of blood platelets (thrombocytes), which are critically involved in blood clotting, are also increased and platelet function can be disturbed. Therefore, also complications due to increased tendency to bleed can arise (bleeding in the gastrointestinal tract and into the skin).
In the case of suspected polycythemia vera (based on unspecific symptoms and changed blood picture, especially increased hemoglobin and hematocrit) patients will be referred to a specialized doctor (hematologist) for further examination.
Important for diagnosis is an initial differentiation from a reactive increase of erythrocytes (meaning that increased red blood cell numbers are due to a different disease).
Therefore, a potential underlying disease of the heart, lung, kidney, or liver needs to be excluded.
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