Polycythemia vera (PV) is a type of blood cancer where the bone marrow makes too many red blood cells. It is a form of chronic leukemia that requires treatment to extend a person’s life.
Polycythemia vera (PV) can be a silent disease. In the early stages, PV can be asymptomatic. Some people find out during a routine blood test that their red blood cell counts are too high, despite not experiencing other symptoms. PV is considered a type of blood cancer due to the abnormal production of red blood cells.
Knowing the symptoms of this rare blood disease is one way to get a diagnosis and receive treatment early.
Many of the symptoms of PV occur due to an excess of red blood cells, which makes the blood thicker than normal. Thicker blood has a difficult time moving through blood vessels. This limits the amount of oxygen that can get to organs and tissue.
Some possible signs and symptoms of PV include:
- blurred or double vision
- headaches
- congestion
- fatigue
- itching
- weight loss
- burning sensation in the skin, especially in the hands and feet
- facial skin redness
- reddish-purplish color on hands and feet
- heavy sweating
These symptoms can also be mistaken for other conditions. As PV progresses, more serious problems can occur, including:
- bleeding gums
- bleeding in the digestive tract
- heavy bleeding from small cuts
- joint swelling
- bone pain
- liver enlargement
- spleen enlargement
- blood clots, or thrombosis
- abdominal pain and fullness
PV can become life threatening when a blood clot cuts off blood and oxygen supply to vital organs. This can cause:
- stroke
- heart attack
- intestinal injuries
- pulmonary embolisms, caused by blood clots in the blood vessels of the lung
Thromboembolic events are a primary complication in people with PV. Thrombosis is when a blood clot forms in either your veins or arteries. Veins are blood vessels that carry blood to your heart. Arteries are blood vessels that carry oxygen-rich blood away from your heart to other parts of your body.
The symptoms of thrombosis depend on the location of the clot. If it forms in the blood vessels of your brain, it can lead to a stroke. If it forms in the blood vessels of your heart, it can lead to a heart attack.
Deep vein thrombosis (DVT) occurs when a blood clot forms in your veins. The most common place for a DVT to develop is in the legs, but it can occur in veins of the arms, abdomen, and pelvis, too. A pulmonary embolism is when a blood clot occurs in your lungs, often from a DVT moving to the lungs from somewhere else in the body.
PV can cause an increase in uric acid. This substance is released when red blood cells are broken down. This can contribute to:
About 10% of those with PV develop myelofibrosis. Myelofibrosis is serious bone marrow scarring in which scar tissue replaces your bone marrow. This scarring can lead to a reduced production of healthy red blood cells.
Myelofibrosis may contribute to an enlarged liver and spleen. Your doctor may recommend a bone marrow transplant for severe cases of PV.
After many years of living with PV, a type of leukemia, some people can develop acute myeloid leukemia or acute lymphoblastic leukemia. Researchers estimate that 4% of people who’ve had PV for more than 10 years will develop acute myeloid leukemia. Acute lymphoblastic leukemia can also occur but is more rare. These conditions require treatments that focus on both PV and the specific type of leukemia.
The key to effectively managing PV is getting treatment early. This can reduce the risk of serious complications, particularly thrombosis.
A variety of treatment options are also available for other symptoms and conditions associated with PV. Talk with your doctor about which medications and treatment options are best for you.
