With treatment, many people with Polycythemia vera (PV) will live at least 14 years after diagnosis. The younger you are, the better your outlook, though other factors can also affect life expectancy.
PV is a rare blood cancer 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. While no cure exists for PV, it can be controlled through treatment, and you can live with the disease for many years.
The exact cause of PV is unknown, but
About 44-57 out of every 100,000 people are affected by the disease. Of these individuals,
The overall median survival period for PV is about
What factors influence outlook?
A few factors can affect your outlook with PV:
- Age: PV is found mostly in older adults and rarely occurs in anyone under the age of 20. That said, for people under age 60, the median survival time is about 24 years, which is significantly higher than the overall rate.
- Treatment: Research shows that the median survival without treatment is 6-18 months, compared to a decade or more with treatment.
- Complications: Your median survival rate is
lower if you experience complications such as blood clots and scarring or if you progress to other types of leukemia.
Your outlook can also be affected by other health conditions and your genetics.
There’s no cure for PV. However, it can be managed.
The main purpose of treatment is to control 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.
Preventing complications
High blood cell counts, no matter the type of blood cell, increase your chance 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.
How serious is polycythemia vera?
Without treatment, PV can be life threatening. The median survival rate after diagnosis is significantly shorter without treatment compared to with treatment.
Is polycythemia vera considered a blood cancer?
PV belongs to a class of blood cancers known as myeloproliferative neoplasms.
How quickly does polycythemia vera progress?
With treatment, many people with PV can lead normal lives. When it does progress despite treatment, it tends to do so slowly. In about a
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.
Consistent monitoring and treatment can help improve your life expectancy and quality of life. Your appointment schedule will depend on your specific case, but it’s generally recommended to see your hematologist every one to three months.