Polycythemia vera (PV) is a rare type of blood cancer. It starts in your bone marrow and causes blood cells to multiply without control. When monitored and properly treated, you can live a normal, long life with the disease.
PV is one of a group of myeloproliferative neoplasm diseases or MPNs. PV starts in your bone marrow where it causes blood cells to multiply with no signal telling them to stop. This causes your blood to thicken.
The thickening of your blood prevents it from flowing freely to your organs and the rest of your body.
The cause of PV is unknown. It can show up at any age, but it’s most often found in those over 60. According to the National Organization for Rare Disorders (NORD), about 2 out of every 100,000 people are diagnosed with PV. Also, PV affects more men than women.
In 2005, researchers found that about 95 percent of people with PV also have JAK2 gene mutation. The JAK2 gene gives directions to make proteins which helps in the growth and division of cells. It also signals when enough cells have been made.
For those with the JAK2 gene mutation, the signal telling the body to stop making cells doesn’t work properly so cells are continuously made.
Researchers still don’t know what causes the JAK2 gene mutation.
The symptoms of PV develop slowly and often don’t show up for years. Early signs and symptoms of PV may include:
- itching after a hot shower
- a red flush in your face
Later, as the disease progresses, your symptoms may become more apparent and include:
- breathing difficulty
- vision problems
For some, the first signs of PV may be problems caused by blood clots.
Because there’s no current cure for PV, treatments are meant to control symptoms and prevent complications. Complications are caused because thickened blood cannot flow freely.
Treatments include the following:
- Phlebotomy: Phlebotomy is a procedure that removes blood from your body to decrease thickness and improve the flow. The procedure is similar to the process you undergo when getting a blood test.
- Aspirin: A low dose of aspirin taken daily can help prevent your blood from clotting.
- Hydroxyurea: Hydroxyurea is a prescription drug that prevents your bone marrow from creating new cells without stopping. After phlebotomy and aspirin, it’s often the first drug given to those with PV.
- Interferon alfa: Interferon alfa is an injectable drug that’s administered with a needle. It’s often given to younger patients or to women who are pregnant.
- Ruxolitinib (Jakafi): Jakafi is a pill known as a JAK2 inhibitor. It targets the JAK2 gene mutations to prevent clotting and to allow the blood to flow more freely. It’s one of the newer forms of treatment for PV.
- Stem cell transplant: If your illness has progressed to a point where drugs and phlebotomy treatments aren’t working, a bone marrow transplant may be considered.
If treated and monitored properly, you can live a normal life with PV, even if you’re over the age of 60. At that point, the disease doesn’t have enough time to reach a stage of complication that could shorten your life.
But the earlier you’re diagnosed the better, as symptoms and complications can be treated or managed.
Talk to Your Doctor
Although PV is a rare disease, it’s not something you should dismiss. If you have any of the symptoms of PV or experience regular blood clotting, make sure to tell your doctor. Staying on top of the disease is one of the best ways to manage and control it.