Polycythemia vera is a type of cancer that causes red blood cells to multiply. This can cause blood to flow more slowly and lead to complications, including blood clots.
There’s no cure for PV, but you can manage the condition with treatment. Your doctor will likely do routine blood draws and prescribe medication to help prevent serious blood clots. It’s important to talk with your doctor if you’re at risk of PV and have any of its symptoms.
Read on to learn about PV, what causes it, how to recognize it, and how it’s treated.
Polycythemia vera (PV) is a rare type of blood cancer in which your body produces too many red blood cells.
Red blood cells carry oxygen throughout your body. When you have too many red blood cells, your blood thickens and flows more slowly. The red blood cells can clump together and form clots inside your blood vessels.
If it’s not treated, PV can lead to life-threatening complications. Blood that flows more slowly can reduce the amount of oxygen that reaches your heart, brain, and other vital organs. Blood clots can completely block blood flow within a blood vessel, causing a stroke or even death.
In the long term, PV can lead to scarring of the bone marrow, known as myelofibrosis, as well as leukemia, another type of blood cancer.
PV might not cause any symptoms for many years. When symptoms first start, they can be mild enough to miss. You might not realize you have PV until a routine blood test picks up the problem.
Recognizing the symptoms early can help you get treatment started and hopefully prevent blood clots and their complications.
- trouble breathing when you lie down
- trouble concentrating
- unintended weight loss
- pain in your abdomen
- feeling full easily
- blurred or double vision
- heavy sweating
- bleeding or bruising
As the disease progresses and your blood becomes thickened with more red blood cells, more serious symptoms can appear, such as:
- heavy bleeding from even minor cuts
- swollen joints
- bone pain
- reddish color to your face
- bleeding gums
- burning feeling in your hands or feet
Most of these symptoms can be caused by other conditions as well, so getting a proper diagnosis from your doctor is critical.
Polycythemia vera occurs more often in men than in women. You’re more likely to get PV after age 60, but it can start at any age.
Mutations (changes) to the JAK2 gene are the main cause of the disease. This gene controls the production of a protein that helps make blood cells. About 95% of people with PV have this type of mutation.
The mutation that causes PV can be passed down through families. But more often, it can happen without any family connection. Research is ongoing into the cause of the genetic mutation behind PV.
If you have PV, your risk for developing serious complications depends on how likely you are to develop a blood clot. Factors that can increase your risk of developing blood clots in PV include:
- a history of blood clots
- being over age 60
- high blood pressure
- high cholesterol
Blood that’s thicker than normal can always increase your risk of blood clots, no matter the cause.
If you think you might have PV, your doctor will first do a test called a complete blood count (CBC). A CBC measures the following factors in your blood:
- the number of red blood cells
- the number of white blood cells
- the number of platelets
- the amount of hemoglobin (a protein that carries oxygen)
- the percentage of space taken up by red blood cells in the blood, known as the hematocrit
If you have PV, you’ll likely have a higher-than-normal amount of red blood cells and hemoglobin, and an abnormally high hematocrit (the proportion of your blood volume taken up by red blood cells). You may also have abnormal platelet counts or white blood cell counts.
If your CBC results are abnormal, your doctor will likely check your blood for the JAK2 mutation. Most people with PV test positive for this type of mutation.
Along with other blood tests, you’ll likely need a bone marrow biopsy to confirm a diagnosis of PV.
If your doctor tells you that you have PV, keep in mind that the sooner you know, the sooner you can start treatment. And treatment reduces your risk of complications from PV.
PV is a chronic condition that doesn’t have a cure. However, treatment can help you manage its symptoms and help prevent complications. Your doctor will prescribe a treatment plan based on your risk of developing blood clots.
Treatment for people with a low risk of blood clots
Typical treatment for those at low risk of blood clots includes two things: Aspirin and a procedure called phlebotomy.
- Low-dose aspirin: Aspirin affects the platelets in your blood, decreasing your risk of forming blood clots.
- Phlebotomy: Using a needle, your doctor will remove a small amount of blood from one of your veins. This helps reduce your red blood cell count. You’ll typically have this treatment about once a week, and then once every few months until your hematocrit level is closer to normal.
Treatment for people with a high risk of blood clots
In addition to aspirin and phlebotomy, people at high risk of blood clots may require more specialized treatment, such as other medications. These can include:
- Hydroxyurea (Droxia, Hydrea): This is a cancer drug that prevents your body from making too many red blood cells. It reduces your risk of blood clots. Hydroxyurea is used off-label to treat PV.
- Busulfan (Myleran): This cancer drug is approved to treat leukemia, but it can be used off-label to treat PV.
- Ruxolitinib (Jakafi): This is one of two drugs
approved by the Food and Drug Administration (FDA)to treat PV. Your doctor may prescribe this drug if you can’t tolerate hydroxyurea or if hydroxyurea doesn’t lower your blood count enough. Ruxolitinib works by inhibiting growth factors responsible for creating red blood cells and immune system functioning.
- Ropeginterferon alfa-2b-njft (Besremi): The second drug
approved in 2021to treat PV is a special form of interferon-alpha. Forms of interferon-alpha have been used off-label to treat PV prior to this FDA approval. It helps your immune system fight off the overactive bone marrow cells that are part of PV. It can also block your body from making too many red blood cells.
Your doctor may also prescribe other treatments for you. Some of these may help relieve itching, which can be a persistent and bothersome problem for many people with PV. These treatments may include:
- selective serotonin reuptake inhibitors (SSRIs)
- phototherapy (treatment with ultraviolet light)
Your doctor will talk with you about the best treatment options for you.
Making a few changes to your lifestyle might help you cope with symptoms and reduce your chance of complications.
That said, it’s important to speak with your doctor first to make sure these changes are right for you, as every person’s needs are different.
The changes include:
- eating a balanced diet to promote healthy weight, cholesterol, and blood pressure levels. This means eating healthy means eating nutrient-dense and balanced meals with lots of fresh fruits and vegetables, whole grains, lean protein, and low-fat dairy will help manage your condition.
- getting regular physical exercise
- limiting or quitting smoking
- managing co-existing conditions like diabetes
Note that eating high-sodium foods can cause your body to shift water into your body’s tissues, which can make some of your PV symptoms worse.
Also, drinking enough fluids will help you avoid dehydration and maintain good blood flow and circulation. Your doctor or a registered dietitian can provide individualized guidance on diet and water intake.
Treating PV helps reduce your risk of life-threatening complications. These include:
- Myelofibrosis, the advanced stage of PV scars the bone marrow and can enlarge the liver and spleen
- heart attack
- deep vein thrombosis (DVT) or a blood clot in the deep vein
- ischemic stroke, caused by loss of blood supply to the brain
- pulmonary embolism or a blood clot in the lung
- hemorrhagic death, which is death from bleeding, usually from the stomach or other parts of the digestive tract
- portal hypertension or increased blood pressure in the liver that can lead to liver failure
- acute myeloid leukemia (AML), which is a particular type of blood cancer that affects white blood cells
While everyone’s situation with PV is different, many people who stick to their treatment plan and see their hematologist regularly can expect to live a long life with limited complications.
That said, treatment is critical. People without any treatment can typically expect to survive
Your life expectancy also depends on whether or not you develop any complications, and these complications are possible even with treatment, though the risk is much lower. Overall, people who receive treatment have a much better outlook than those without it.
For people with PV, about a
Polycythemia vera is a rare blood disease that increases your risk of dangerous blood clots and other complications. It’s not curable, but it’s treatable. New diagnostic and treatment options are being researched.
If you have polycythemia vera, talk with your primary care doctor or a hematologist about the right treatment plan for you. This will likely include phlebotomy and medications.
Getting the care you need as soon as possible can help prevent blood clots, decrease complications, and improve the quality and length of your life.