Causative and risk factors
Polycythemia vera occurs due to a genetic mutation in the cells of the bone marrow. However this condition is not inherited. Thus one can suffer from polycythemia vera, even though none of the parents have this condition.
Clinical Presentation
Many patients of polycythemia vera are elderly, beyond the age of 60 years.
Most people with this condition remain asymptomatic. However some patients may develop certain non-specific symptoms such as headache, fatigue, profuse perspiration, dizziness, breathlessness etc. The patient may experience itching of the skin, especially after a warm bath. The great toe may be swollen and there may be tingling, numbness and weakness in the extremities.
Since the excess of blood cells causes the blood to thicken, certain complications may arise such as pulmonary embolism, deep vein thrombosis and splenomegaly. Polycythemia vera may also progress to other conditions like myelodysplastic syndrome, myelofibrosis or acute leukemia.
Investigations
A complete blood cell count is advised along with examination of a peripheral blood smear. The hemoglobin level and the red cell count are found to be high. The hematocrit measurement is elevated. The levels of erythropoietin hormone are found to be low. Most patients are advised to undergo a bone marrow biopsy examination. Genetic testing may be performed on a sample of blood or bone marrow.
Treatment
Polycythemia vera is an incurable condition, the treatment aims at preventing complications.
A procedure known as phlebotomy is done in which blood is removed from the veins. This procedure is beneficial to most patients. Medications include the use of blood thinners, chelating agents and antihistamines. Ultraviolet light treatment is recommended to relieve the itching.
Certain lifestyle changes are advised to the patient. These include avoidance of extreme temperatures, restriction of tobacco intake and regular moderate exercise.