The symptoms of polycythemia vera come on slowly. Because there are too many red blood cells, there is more blood in the body than normal, it is thicker than normal and has difficulty circulating in small blood vessels. Symptoms may include:
- poor oxygen circulation - headache, dizziness, vertigo, ringing in the ears (tinnitus), vision changes, chest pain
- abnormal bleeding - nosebleeds, gums bleeding, bruising, digestive system bleeding
- enlarged spleen (splenomegaly) and/or liver (hepatomegaly)
- abnormal blood clots in veins in the body (thrombosis)
- may develop Budd-Chiari syndrome (obstruction of the main vein of the liver)
- itching (pruritis), especially after a hot bath
- ruddy (reddened) complexion - may be seen in the face, palms, nailbeds, mucous membranes in the mouth, and conjunctiva of the eye
When polycythemia vera is suspected, perhaps from a high blood hematocrit or other symptoms, direct measurement of the red blood cell mass in the blood will show an increase. This combined with an enlarged spleen or increased white blood cell and platelet counts helps confirm the diagnosis. There are additional tests which can be done to confirm the diagnosis and exclude other disorders which may cause similar symptoms.
Polycythemia vera has for many years been treated by removing some blood from the body (phlebotomy). This reduces the increased number of red blood cells down to a normal level. Usually about one-half to one pint (250-500 ml) of blood is removed. When an individual is first diagnosed, phlebotomy may be done frequently.
Once the individual's hematocrit value is normal, phlebotomy may be done on a maintenance basis, or may be combined with, or replaced by, myelosuppressive drugs such as hydroxyurea or interferon. If the platelet count is high, anagrelide (Agrylin) may be used.
An individual with polycythemia vera will be cared for by a hematologist (blood specialist) who has experience managing the treatment and complications of the disorder. There is as yet no consensus in the medical community as to what the optimum treatment for PV is, so treatment is tailored to the needs of the individual. PV is a chronic condition which requires long-term treatment.
- Besa, E.C. (2006). Polycythemia vera. eMedicine, accessed at http://www.emedicine.com/med/topic1864.htm
- CMPD Education Foundation. Frequently Asked Questions About Polycythemia Vera
- National Organization for Rare Disorders. Polycythemia Vera