Polycythemia vera (PV) is a rare blood cancer. While no cure exists for PV, it can be controlled through treatment, and you can live with the disease for many years.
PV is caused by a mutation or abnormality in the genes of the stem cells in your bone marrow. PV thickens your blood by producing too many red blood cells, which can block blood flow to organs and tissues.
The exact cause of PV is unknown, but
PV is found mostly in older adults. It rarely occurs in anyone under the age of 20.
About 2 out of every 100,000 people are affected by the disease. Of these individuals,
The main purpose of treatment is controlling your blood cell counts. Reducing the number of red blood cells helps prevent clots that could lead to stroke, heart attack, or other organ damage. It may also mean managing white blood cell and platelet counts. The same process that signals the overproduction of red blood cells seems to also signal the overproduction of white blood cells and platelets. High blood cell counts, no matter the type of blood cell, increase the risk of blood clots and other complications.
During treatment, your doctor will need to monitor you regularly to watch for thrombosis. This occurs when a blood clot develops in an artery or vein and obstructs the flow of blood to your major organs or tissues.
A long-term complication of PV is myelofibrosis. This occurs when your bone marrow is scarred and can no longer produce healthy cells that function properly. You and your hematologist (a specialist in blood disorders) may discuss having a bone marrow transplant depending on your case.
Leukemia is another long-term complication of PV. Specifically, both acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL) are associated with polycythemia vera. AML is more common. You may need specialized treatment that also focuses on leukemia management if this complication develops.
PV is rare, so regular monitoring and checkups are important. When you’re first diagnosed, you may want to seek out a hematologist from a major medical center. These blood specialists will know more about PV. And they likely have provided care for someone with the disease.
Once you find a hematologist, work with them to set up an appointment schedule. Your appointment schedule will depend on the progression of your PV. But you should expect to see your hematologist about once a month to once every three months depending on blood cell counts, age, overall health, and other symptoms.
Regular monitoring and treatments can help maximize your life expectancy and improve your overall quality of life. Depending on a wide range of factors, current life expectancy has been shown to be