Polycythemia vera (PV) is a chronic blood malignancy caused by an acquired gene mutation of the blood-producing cells in the bone marrow which causes 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). Conversion to myelofibrosis or, in rare cases, acute leukemia can occur as the disease progresses over time.
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.
PV cannot be prevented. Research is underway to learn more about how the disease develops.
PV is a chronic disease, but it can be effectively managed. PV can cause different symptoms in each patient. In some cases when symptoms may not be present, treatment may not be required for extended periods of time, although routine monitoring with a physician is recommended for all PV patients. One of the main goals of monitoring and treatment is to reduce the risk of blood clotting. Treatment options include phlebotomy (removal of blood), low-dose aspirin, and drug therapies such as hydroxyurea, anagrelide, ruxolitinib, or interferon alfa. Antihistamines and avoidance of hot showers can help with itching. Other treatment options may be available through clinical trials.
Sources: Leukemia and Lymphoma Society, MPN Research Foundation, and National Cancer Institute
