Polycythemia vera (PV) is a rare, chronic form of blood cancer. While there’s no cure, PV can be controlled through treatment and you can live with the disease for many years.
PV is a mutation or abnormality in the stem cells in your bone marrow. It makes your blood thicken by producing too many red blood cells that may block blood flow to the rest of the body.
The exact cause of PV is unknown, but 95 percent of those who have the disease also have the JAK2 genetic mutation. This genetic mutation can be detected in a regular blood test.
PV is found mostly in older individuals, and it rarely targets anyone under the age of 20.
Only about 2 out of every 100,000 people are diagnosed with the disease every year. Out of these individuals, only 15 percent ever develop an advanced, serious stage of PV.
The main purpose of treatment is controlling your PV symptoms. This means reducing the number of red blood cells to prevent clots that could lead to stroke or heart attack. It may also mean reducing white blood cell and platelet counts.
During treatment, you’ll need to be monitored regularly to watch for arterial thrombosis, which is when a blood clot develops in an artery and obstructs the flow of blood to your major organs.
The advanced stage of PV is called myelofibrosis. At this stage, your bone marrow can no longer produce healthy cells that function properly. You and your hematologist may discuss having a bone marrow transplant, but this is often the last treatment option.
Monitoring and Outlook
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 may have even cared for someone with the disease.
Once you find a hematologist you will 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.
With regular monitoring and treatments, people have been able to live with PV for 30 to 40 years.