Polycythemia Vera (PV) is a chronic blood malignancy caused by an acquired gene mutation of the blood-producing cells in the bone marrow, which creates an overproduction of blood cells, particularly red blood cells. White blood cells and platelets are often also increased. An excess amount of red blood cells can increase the blood viscosity (thickness), slowing blood flow. As blood flow is slowed, the risk of blood clotting increases. PV can also cause the spleen to become enlarged. PV is one of a group of rare bone marrow cancers called myeloproliferative neoplasms (MPNs) that develop due to an acquired mutation in the DNA of the stem cells in the bone marrow. Conversion to myelofibrosis or acute leukemia can occur.
Statistics
- In the United States, for every 100,000 people, there are approximately 1.3 people diagnosed with polycythemia vera annually.
- A majority of polycythemia vera cases are diagnosed in people over the age of 60.
Risk Factors
Symptoms
Many patients with PV do not have symptoms when they are diagnosed. Diagnosis often occurs during a routine exam or blood test. However, people may experience persistence of any of the following symptoms.
- Headaches
- Sweating and night sweats
- Ringing in the ears
- Blurred vision or blind spots
- Dizziness or vertigo
- Skin with red or purple hue
- Bleeding or clotting excessively
- Reddened face
- Unexpected weight loss
- Feeling full quickly on eating
- Abnormal discomfort on left side below the ribs
- Itching, especially after a shower or bath
- Burning and redness of the hands or feet
- Fatigue
- Bone pain
- Gout attacks
- Shortness of breath
- Weakness
- Bruising excessively
- Numbness or tingling in feet
Prevention
PV cannot be prevented. Research is underway to learn more about how the disease develops.
Treatment
PV is a chronic disease, but can be managed well. PV causes differing reactions in each patient. In some cases, symptoms may not be present, in which case treatment may not be required. However, treatment options usually include phlebotomy (removal of blood), and low-dose aspirin. Drug therapy such as hydroxyurea, anagrelide, ruxolitinib, and interferon are also options. Antihistamines and avoidance of hot showers can help with itching. Clinical trials can also be an important treatment option.