What Is Polycythemia Vera?
Polycythemia vera (PV) is a blood cancer that begins in the marrow of your bones, the soft center where new blood cells grow. It causes your marrow to make too many red blood cells so your blood is too thick. You may be more likely to have clots, a stroke, or a heart attack.
This disease gets worse slowly, usually over many years. It can be life-threatening if you don't get treatment, but the right care can help you live a long life.
Most people who have PV don’t get diagnosed until they’re 60 or older, usually after a routine blood test. But it can happen at any age. Men get it more often than women.
It's common to worry when you find out that you have cancer. Remember that everyone is different and that all cancers aren’t the same. With the support of your doctor, family, friends, and other people who have polycythemia vera, you’ll be in the best position to manage it.
Polycythemia Vera Causes
Your bone marrow makes three types of blood cells:
- Red
- White
- Platelets
Red blood cells carry oxygen, white ones fight infections, and platelets clot your blood to stop bleeding.
Most people with polycythemia vera have too many red blood cells. But it can also cause you to have too many white blood cells and platelets.
PV is caused by a gene (either JAK2 or TET2) that doesn’t work the way it should. Most likely, the problem develops over the course of a person's life.
Is polycythemia vera hereditary?
PV is usually not inherited; however, parents can pass these affected genes to children. In rare cases, polycythemia vera risk may run in families.
Polycythemia Vera Symptoms
At first, you may not notice any problems. When they do show up, polycythemia vera symptoms can include:
- Headaches
- Double vision
- Dark or blind spots in your vision that come and go
- Itching all over your body, especially after you've been in warm or hot water
- Sweating, especially at night
- A red face that looks like sunburn or blushing
- Weakness
- Dizziness
- Weight loss
- Shortness of breath
- Tingling or burning in your hands or feet
- Painful joint swelling
You may also feel pressure or fullness below your ribs on your left side. This might mean PV has enlarged your spleen, an organ that helps filter your blood.
Polycythemia vera leg pain
Blood clots are one possible complication of polycythemia vera. When a blood clot forms in the legs, it's called deep vein thrombosis (DVT). This can cause pain and swelling in the legs.
Polycythemia vera end-stage symptoms
At the end stage, also known as the "spent phase," of the disease, your bone marrow stops making healthy blood cells. This happens because the abnormal blood cells overtake your bone marrow, where they break down and are replaced with scar tissue.
Too much scar tissue can prevent your bone marrow from doing its job: making healthy red blood cells. This can increase your risk of anemia. You may need blood transfusions to help make sure your body has enough healthy blood cells. The spleen can become swollen and painful during this stage and may need to be removed through surgery.
Polycythemia Vera Complications
If left untreated, PV can become deadly.
It can cause blood clots , which can lead to a heart attack, a stroke, a pulmonary embolism, or deep vein thrombosis (DVT). They might also make your liver or spleen larger. If your organs don’t get enough blood, you could have chest pain or heart failure.
Having too many red blood cells might cause stomach ulcers, gout, or kidney stones. PV can also lead to more serious blood diseases such as acute leukemia or myelofibrosis.
Polycythemia vera blood clots
If you have PV, there are too many red blood cells trying to move through your body. This makes your blood thicker, causing it to flow more slowly. Your platelets — blood cells that help with clotting — can also become irregular. All of this makes blood clots more likely to form, which in turn can lead to other dangerous complications, including stroke, heart attack, and organ damage. Blood clots can also block arteries in your lungs, leg muscles, or abdomen.
Polycythemia vera and leukemia
In about 3% of people with polycythemia vera, the disease changes into acute myeloid leukemia. Leukemia happens because the bone marrow makes immature cells too quickly. These cells can spread throughout the body and block healthy blood cells from growing.
Polycythemia vera and myelofibrosis
Myelofibrosis is a type of blood cancer that's very similar to the end stages of polycythemia vera. It happens because too many abnormal cells break down in the bone marrow, replacing bone marrow with scar tissue. The abnormal cells can also reach other organs in your body. About 5%-6% of people with polycythemia vera develop myelofibrosis within the first 10 years of being diagnosed.
Polycythemia Vera Diagnosis
Your doctor will start with a physical exam, including a check of your spleen. They’ll ask about your symptoms and see if your face is unusually red.
Polycythemia vera tests
A few different tests can help determine if you have polycythemia vera.
Blood tests. Blood tests can check if your red blood cell count is high. An unusually high number of any of these could be a sign of polycythemia vera.
Your doctor might also test your blood for a hormone called erythropoietin, which helps your body produce red blood cells. People who have polycythemia vera have very low amounts of it.
Molecular testing. This uses a sample of your blood, tissue, or body fluid, which can show a JAK2 gene mutation linked to polycythemia vera.
Bone marrow biopsy
Based on your blood and molecular tests and symptoms, your doctor might want to do a bone marrow biopsy. This test collects small amounts of solid and liquid bone marrow through a thin needle. The sample is usually taken from the back of your hip, while you lie on your stomach. Your doctor will use local anesthesia and possibly light sedation.
The results can show your doctor if your bone marrow makes too many blood cells.
Questions for your doctor
It's normal to feel nervous before and during a doctor's appointment. To ease your mind, make a list of questions beforehand and bring it with you. This can also ensure that you don't forget what you wanted to ask your doctor. Here are some questions you may want to ask:
- What tests will I need?
- Does polycythemia get better or go away?
- What's causing my condition and symptoms?
- Will I need treatment?
- What are my treatment options?
- Do I need to see a specialist?
- What are the side effects?
- How can I prevent complications?
- Since I have polycythemia vera, am I more likely to have a stroke or heart attack?
- How can I ease my symptoms?
Polycythemia Vera Treatment
If you don't have many PV symptoms, you might not need treatment right away. Your doctor will keep a close watch on you.
If you do need treatment, it will be aimed at lowering the amount of red blood cells your body makes and preventing blood clots and other complications.
Your options include:
This is often the first treatment for people with polycythemia vera.
Phlebotomy is just like donating blood. Blood is taken from a vein to help reduce the volume of blood and the number of blood cells in your body. This makes your blood thinner, preventing it from clumping in blood vessel walls. Phlebotomy can help relieve symptoms such as headaches, vision problems, itchiness, dizziness, and ringing in the ears.
Your doctor will decide how often you need phlebotomy. Some people with PV don’t need any other treatment for many years.
Low-dose aspirin
Your doctor might prescribe a low dose of aspirin (usually 81 milligrams). Aspirin can help stop platelets from sticking together and forming clots. This can help prevent blood clots, heart attacks, and strokes. Aspirin can cause side effects such as stomach upset and heartburn.
Most people with polycythemia vera take low-dose aspirin.
Medicine to lower blood cells
If you have polycythemia vera, your doctor might prescribe medicines to help lower the amount of blood cells in your body. These include:
Busulfan. You might take busulfan (Busulfex, Myleran) or ruxolitinib (Jakafi) if hydroxyurea isn’t helpful or if it causes severe side effects. It can bring your blood cell counts back to normal and help control the JAK2 mutation.
Hydroxyurea (Droxia, Hydrea). This medicine limits how many blood cells your bone marrow produces.
Interferon alfa. Interferon alfa (Intron A) or ropeginterferon alfa-2b-njft (Besremi) is injected. It may help control the JAK2 mutation and get blood cell counts back to normal. It can be taken early in the disease to prevent it from progressing.
Ruxolitinib. This is a type of kinase inhibitor, a medicine that blocks enzymes linked to certain cancers. Many people with polycythemia vera have a mutation in the JAK2 protein. Ruxolitinib causes changes to JAK2 and JAK1, which helps curb the production of red blood cells. It’s an alternative option to hydroxyurea. It may be prescribed for secondary myelofibrosis.
If you have itching that doesn't go away, your doctor may give you antihistamines.
Lifestyle Changes
There are things you can do every day to help manage polycythemia vera symptoms.
Stay hydrated. This is even more important if you have thicker blood.
Stop smoking. Smoking increases your risk of blood clots.
Exercise. Exercise can help boost your fitness and mood, keep your heart healthy, and help your circulation. If you have fatigue, start with gentle exercise, such as walking, yoga, or gardening. Also, doing leg and ankle exercises can help keep clots from forming in the veins of your legs.
Manage itchiness. Itching is common in people with polycythemia vera. It can help to take warm or cool showers, instead of very hot showers. Be sure to pat your skin dry, rather than rubbing dry.
Eat a balanced diet. Make sure to eat enough healthy carbohydrates, lean protein, fresh fruits and vegetables, dairy products, fiber, and healthy fats. Iron-rich foods, such as liver, are also recommended for polycythemia vera.
Bathe at the right temperature. Bathe or shower in cool water if warm water makes you itch.
Keep your skin moisturized. Use hydrating lotion, and try not to scratch.
Polycythemia Vera Prognosis
There's no cure, but the right treatment can help you manage this disease for many years.
Talk to your friends and family about what you’re dealing with. You may also want to join a support group for people who’ve been in your position.
The MPN Research Foundation has more information about polycythemia vera. It can also help you find support groups.
Takeaways
Polycythemia vera is a blood cancer that causes your bone marrow to make too many red blood cells. It can cause complications like blood clots and anemia, but treatments are available. In rare cases, polycythemia can change into other diseases, such as leukemia and myelofibrosis.
Polycythemia Vera FAQs
What is the life expectancy with polycythemia vera?
The average life expectancy with polycythemia vera is 20 years after diagnosis.
Is polycythemia vera cancer curable?
There’s no cure for polycythemia vera, but it can be managed with medication.
What does PV itching feel like?
You might feel itchy after taking a hot shower or bath. PV can also cause your face to redden and feel a burning sensation.
What are the final stages of polycythemia vera?
In the final stages of polycythemia vera, your bone marrow stops producing healthy cells and is replaced with scar tissue.
