Polycythemia vera (PV) is a slow-growing blood cancer that can lead to complications such as blood clots, bleeding, and an enlarged spleen. In rare cases, it can progress to other blood cancers, which can be life threatening.

PV causes excess red blood cell production. It can also increase the amount of white blood cells and platelets in the blood. The extra cells make the blood thicker and more likely to clot.

PV increases the risk of acute myeloid leukemia, myelofibrosis, and myelodysplastic syndromes. These are rare but potentially serious complications.

There is no cure for PV, but treatments are available to manage symptoms and thin the blood. This helps reduce the risk of a blood clot or the following complications:

Deep vein thrombosis (DVT) can happen when a blood clot forms in a large, deep vein in the body. People with PV have a higher chance of getting a DVT because the blood flows more slowly and clots easier.

The symptoms of DVT include swelling, pain, cramping, and red or warm skin. If the blood clot travels to the lung, you can get a pulmonary embolism (PE), which is life threatening.

Signs of PE include difficulty breathing, chest pain, rapid heart rate, and dizziness. Get medical help immediately if these symptoms occur, even if you don’t have leg pain.

Some people with PV also produce too many platelets in their blood. Platelets (thrombocytes) play a crucial role in clotting and preventing bleeding, but with PV, there can be an excess amount that doesn’t function properly.

As a result, you can experience bleeding issues such as bleeding gums, ulcers, nosebleeds, and bruising or pooling of blood under the skin.

When histamine rises in your body, it may also affect your stomach. Specifically, your stomach may produce more acid, which can lead to the formation of peptic ulcers.

Peptic ulcers cause stomach pain, as well as feeling bloated or nauseated. In some cases, it can lead to abdominal bleeding.

Fortunately, there are medicines that can reduce stomach acid and control ulcer formation.

Learn about peptic ulcer treatment.

Your spleen recycles your blood, but if you have PV, it needs to work harder to remove excess blood cells, leading to an increase in its size.

This is called splenomegaly and affects 30-40% of people with PV. Your treatment options may include medication or surgery to remove the spleen.

Learn about the common symptoms of an enlarged spleen.

About 31–69% of people with PV experience itchy skin, also called pruritus, especially after contact with warm water.

This is likely caused by extra red blood cells triggering the release of a chemical called histamine by the immune system. This is the same chemical that’s released when you have an allergic reaction.

Some tips that can help you find relief include taking a cool shower or bath, avoiding rubbing your skin, and your skin moisturizing daily.

In some cases, your doctor may recommend that you take an antihistamine or a low dose aspirin to help reduce the itching.

Your blood is responsible for carrying oxygen throughout your body. However, when PV slows down your blood flow, it becomes difficult for oxygen to reach all your organs.

This can lead to symptoms such as fatigue, weakness, headaches, dizziness, shortness of breath, ringing in the ears, flashes of light in your vision, and chest pain.

Fortunately, PV treatments can help your blood oxygen move more freely and reduce these symptoms.

Learn more about low blood oxygen (hypoxemia).

PV causes a high turnover of red blood cells, which can increase the production of uric acid. This can lead to gout, which is caused by too much uric acid in your joints.

Gout is a type of arthritis that causes the formation of hard crystals that cause pain and inflammation. This can also lead to kidney stones.

The symptoms of gout include swelling and discomfort in the joints, particularly in the big toe. The symptoms of kidney stones include burning or pain while urinating, flank pain, needing to use the bathroom a lot, and blood in your urine.

Your doctor may prescribe medications like allopurinol (Zyloprim, Lopurin) to manage gout and prevent future episodes. The treatment for kidney stones depends on the type, but they generally have to be removed or broken down.

PV falls into the category of myeloproliferative neoplasms, which are cancers that develop when the bone marrow makes an abnormal amount of blood cells, affecting the production of red and white blood cells and platelets. These damaged cells don’t function properly and crowd out healthy cells.

In rare cases, PV can transform into myelofibrosis (MF), which is another type of neoplasm that tends to be slow-growing. With MF, scar tissue builds up in the bone marrow. Healthy cell production is blocked by cancer cells and scar tissue. The result is lower levels of white and red blood cells and platelets.

In some cases, PV can also transform into acute myeloid leukemia (AML), which is an aggressive cancer and the most common form of leukemia in adults.

AML develops from PV in about 4% of cases over a 10-year period. However, if your PV transforms to MF, you have a 20% chance of it progressing further to AML.

Learn about the outlook for myelofibrosis and AML. Undergoing a stem cell transplant is the only cure for these types of blood cancers. However, this is a procedure posing risks, especially for older adults and people with other health conditions.

In addition, people with PV have a 60% higher chance of progressing to other non-blood-related cancers. This includes skin cancer, prostate cancer, and breast cancer.

What is the most common cause of death in polycythemia vera?

In a 2018 study, researchers found that 41% of subjects with PV died from thrombosis, which is a blood clot-related complication such as DVT.

What is end-stage polycythemia vera?

When PV reaches its final stage, it is considered to be MF. This is also called the “spent phase.”

What should I ask my doctor about polycythemia vera complications?

Tell your doctor if you’re experiencing any symptoms that may be associated with a complication of PV. Some complications may be minor, but others can be fatal without prompt treatment, such as DVT and PE, for example.

With PV, you would typically be treated by a hematologist, who is a doctor specializing in diagnosing and treating blood diseases and disorders.

Here are some questions to ask your hematologist about PV, its symptoms, treatment, and complications.

PV is a type of blood cancer that causes higher than healthy levels of blood cells. Thicker blood is more likely to clot, so treatment is necessary. In rare cases, PV can progress to other types of blood cancers.

There are different treatment options available to help manage symptoms and keep the disease from worsening. Keep your healthcare team up to date on how you are feeling. Regular blood work and appointments will help determine the best care plan for you.