Health

Detailed Information You Need to Know About Polycythemia Vera

March 28, 2022

What is Polycythemia Vera?

Polycythemia Vera (PV) is a rare type of blood cancer in which your body produces too many red blood cells. The bone marrow is responsible for the production of new blood cells. So it begins in the bone marrow and causes it to make too many red blood cells that make your blood too thick.

When your blood becomes thick, it flows more slowly. It causes the red blood cells to clump together and form clots inside your blood vessels. The clots can increase the risk of a stroke or a heart attack.

Polycythemia Vera is a chronic medical condition. If left untreated can lead to life-threatening complications. Red blood cells also carry oxygen to the body. Blood that flows more slowly can reduce the amount of oxygen that reaches your heart, brain, and other vital organs. And blood clots can completely block blood flow within a blood vessel, causing a stroke or even death.

In the long term, Polycythemia Vera can lead to scarring of the bone marrow. It can cause leukemia, another type of blood cancer. There is no cure for Polycythemia Vera. But can be managed with treatment. The doctor advises routine blood draws and prescribes medication to help prevent blood clots. It is crucial to talk to your doctor if you are at high risk of Polycythemia Vera and have any symptoms.

Polycythemia Vera Symptoms

Polycythemia Vera might not cause any symptoms for many years. When symptoms first start, they are not noticeable. You might not recognize you have Polycythemia Vera until a routine blood test picks up the problem.

Initially, the symptoms can help you get treatment started early and hopefully prevent blood clots and their complications. Common symptoms of Polycythemia Vera include:

Dark or blind spots in your vision come and go.
Trouble breathing when you lie down.
Trouble concentrating.
Unplanned weight loss.
Pain in your abdomen.
Feeling easily full.
Blurred or double vision.
Heavy sweating.
Bleeding or bruising.

As the disease progresses and blood becomes thickened with more red blood cells, more chronic 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.

Polycythemia Vera Complication

Without treatment, people with Polycythemia Vera may also be more likely to experience complications, such as:

1- Blood Clots

In Polycythemia Vera, the blood gets thick and can stick together, forming clots inside your veins. It can happen in different places in your body. Like, deep vein thrombosis is a clot formation in a vein deep inside your leg. Sometimes a clot gets loose and travels through a blood vessel. From there, it can move into your lung and get stuck, causing a pulmonary embolism. Also, a clot can lodge in the brain and cause a stroke. Or it can block a blood vessel in the heart and cause a heart attack.

A blood clot can also form in the main blood vessel that leads to the liver. This rare condition is called Budd-Chiari syndrome. Its symptoms may include:

Pain in the upper right part of your belly.
Yellow color in your skin and the whites of your eyes.
Swelling in the stomach or arms.
Bleeding in your digestive tract, from the esophagus or gut.

The person may generally experience the following symptoms due to clot formation.

Chest pain.
Shortness of breath.
Pain and swelling in your leg.

Blood clot formation can be prevented by medication and phlebotomy.

2- Low Oxygen

Red blood cells carry oxygen to all parts of the body. Polycythemia Vera slows the blood flow it reduces the oxygen supply to vital organs. The person may experience the following:

Shortness of breath.
Ringing in your ears.
Vision changes, such as flashes of light.
Chest pain.

Polycythemia Vera treatments can improve blood flow and oxygen transport to prevent these symptoms.

3- Bleeding

Sometimes Polycythemia Vera prompts your body to make extra platelets. Platelets help your blood clot, but the extra ones in Polycythemia Vera prevent your blood from clotting. It causes the person to bleed too easily. They may experience:

Bleeding gums.
Bleeding ulcer or bleeding in the GI tract.
Bruises or pooled blood under the skin.

4- Itching

People with Polycythemia Vera mostly have itchy skin. About 4 out of 10 people have itchy skin. Itching is caused by the extra red blood cells that prompt your immune system to release a chemical called histamine. It makes your skin itch and is released in response to an allergic reaction. To prevent itchiness, you can do the following things:

Keep the water cool when you shower or take a bath.
When you get out of the water, gently pat your skin dry.
Use a moisturizer every day.

Your doctor may recommend an antihistamine or low-dose aspirin.

5- Ulcers

The high histamine levels also cause your stomach to make more acid. The acid can produce sores in the lining of your esophagus, stomach, or small intestine, called peptic ulcers.

The person may experience:

Stomach pain.
A feeling of fullness.
Passing dark, tarry stools.

Doctors recommend proton pump inhibitors or H2 blockers to treat ulcers and prevent new ones.

6- Enlarged Spleen

The spleen recycles old red blood cells. In Polycythemia Vera, the spleen works hard and removes all the extra blood cells. All that extra work makes the spleen grow bigger. It is called splenomegaly. The person may experience symptoms like:

A feeling of fullness.
Swelling in your belly.
Weight loss.
Stomach pain.

The doctor recommends medicine or surgery to remove the enlarged spleen.

7- Gout

Gout is arthritis caused by the buildup of uric acid in joints. Uric acid forms into hard crystals that cause sore and swollen joints. In Polycythemia Vera, cells turn over too quickly, which is the primary cause of gout. The person may suffer from pain and swelling in joints, especially the big toe. The doctor can prescribe medicines such as allopurinol to control gout.

8- Myelofibrosis and Leukemia

After years of pumping out extra red blood cells, the bone marrow can become filled with scar tissue. The bone marrow can produce enough blood cells to meet the body's needs. This condition is called myelofibrosis. It is a rare condition in which abnormal bone marrow cells grow out of control. It can lead to blood and bone marrow cancer is called acute myelogenous leukemia.

Polycythemia Vera Causes

Polycythemia Vera occurs more often in men than in women. Doctors classify Polycythemia Vera into 2-types based on the cause of the condition.

Primary Polycythemia Vera: occurs due to genetic mutation (abnormality). All people diagnosed with primary Polycythemia Vera were found to have a mutation in the JAK2 (Janus kinase) gene. In most cases, Polycythemia Vera is not hereditary (passed down through families), except for a few. Also, found mutations in the TET2 gene in polycythemia Vera cells.

Secondary Polycythemia Vera (acquired): occurs in people who experience low oxygen levels in their blood for a long time. This extended lack of oxygen causes the body to make extra amounts of a hormone called erythropoietin (EPO). A high level of EPO in the body can cause it to make too many red blood cells.

Polycythemia Vera Risk Factors

The disease affects people of all ages but is most common in adults older than 60. Polycythemia Vera is rare in children and young adults. Men are at slightly higher risk for Polycythemia Vera than women. Factors that increase your risk of developing blood clots in Polycythemia Vera include:

A history of blood clots.
Being over age 60.
High blood pressure.
High cholesterol.

Polycythemia Vera Diagnosis

Blood Test

The doctor will first do a complete blood count (CBC) test if a person experiences signs and symptoms of Polycythemia Vera. 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 is known as the hematocrit.

In Polycythemia Vera, the blood test shows a high amount of red blood cells, hemoglobin, and abnormally high hematocrit. Also may have abnormal platelet counts or white blood cell counts.

Bone Marrow Biopsy

The doctor may recommend a bone marrow biopsy to confirm a diagnosis of Polycythemia Vera. A bone marrow biopsy involves taking a small sample of the bone marrow with a needle for examination under a microscope.

Genetic Tests

Although genetic causes of Polycythemia Vera are rare, the doctor performs a genetic test if CBC results are abnormal. They may recommend examining cells in the blood for JAK2 mutation. Most people with Polycythemia Vera tested positive for this type of mutation. After the diagnosis, the person starts the treatment sooner to prevent complications.

Polycythemia Vera Treatment

Polycythemia Vera is a chronic condition that does not 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 low-risk people

The doctor recommends the following treatment for low-risk blood clots. It 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. It helps thin the blood.
The doctor will remove a small amount of blood from one of your veins using a needle. It helps reduce the red blood cell count. The person has to take this treatment about once a week. And then once every few months until your hematocrit level is closer to normal.

Treatment for high-risk people

People at high risk of blood clots may require more specialized treatment. The doctor recommends some additional medications with aspirin and phlebotomy. These can include:

Hydroxyurea (Droxia, Hydrea) is a cancer drug that prevents your body from making too many red blood cells. It helps reduce the risk of blood clots. And is an off-label medicine used to treat Polycythemia Vera.
Interferon-alpha: This drug helps your immune system fight off the overactive bone marrow cells. It can also block your body from making too many red blood cells. Like hydroxyurea, interferon-alpha is an off-label medicine used to treat PV.
Busulfan (Myleran): This cancer drug is approved for leukemia and is an off-label medicine used to treat PV.
Ruxolitinib (Jakafi): The only drug approved by the U.S. Food and Drug Administration to treat Polycythemia Vera. The doctor may prescribe this drug if the patient cannot tolerate hydroxyurea. Or if hydroxyurea does not lower your blood count enough. Ruxolitinib works by inhibiting growth factors that create red blood cells and immune system functioning.

Related treatments

Your doctor may also prescribe other treatments to treat the symptoms. Some may help relieve itching, a persistent and bothersome problem for many people with Polycythemia Vera. These treatments may include:

Selective serotonin reuptake inhibitors (SSRIs).
Phototherapy (treatment with ultraviolet light).

The doctor will talk with the patient about the best treatment options.

Polycythemia Vera Diet

In general, the diet recommended for people with Polycythemia Vera is almost the same for other people. The patient can eat well-balanced meals with fresh fruits and vegetables, whole grains, lean protein, and low-fat dairy. Also, the doctor recommends how many calories should consume each day to maintain a healthy weight.

Reduce salt and keep a check on salt consumption. 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, drink enough fluids to avoid dehydration and maintain good blood flow and circulation. The doctor can provide individualized guidance on diet and water intake.

Polycythemia Vera Prognosis

The prognosis of Polycythemia Vera depends basically on the treatment. Treatment helps reduce your risk of life-threatening complications, such as:

Myelofibrosis: the advanced stage of PV that scars the bone marrow and can enlarge the liver and spleen.
Heart attack.
Deep vein thrombosis (DVT).
Ischemic stroke: a stroke caused by loss of blood supply to the brain.
Pulmonary embolism: a blood clot in the lung.
Hemorrhagic death: death from bleeding, usually from the stomach or other parts of the digestive tract.
Portal hypertension: increased blood pressure in the liver can lead to liver failure.
Acute myeloid leukemia (AML) is a type of blood cancer affecting white blood cells.

These complications are possible in Polycythemia Vera, even with treatment. 5 to 15 percent of people with Polycythemia Vera may develop myelofibrosis 15 years after diagnosis. And less than 10 percent have typically developed leukemia 20 years after diagnosis. Overall, people who receive treatment have a much better condition than those without it.

In addition, taking care of overall health can lower the risk of blood clots from Polycythemia Vera. The patient may take crucial steps to improve the conditions, such as:

Quit smoking.
Staying physically active.
Managing other health conditions such as diabetes, high blood pressure, and heart disease.

Polycythemia Vera Life Expectancy

In Polycythemia Vera, the condition of every individual is different. But many people who stick to their treatment plan and see their hematologist regularly can expect to live a long life with fewer complications.

The treatment of Polycythemia Vera is crucial. Depending on their age and overall health, people without treatment typically expect to survive less than two years. But those who have treatment may live longer. The average length of survival after diagnosis is at least 20 years.

Lifestyle and Home Remedies

Several lifestyle changes can make life better for patients with Polycythemia Vera. These include:

Moderate exercise: Walking is recommended to improve your blood flow. It helps decrease the risk of blood clots. Also, Leg and ankle stretches can improve your blood circulation.
Avoid tobacco: Using tobacco can cause your blood vessels to narrow, increasing the risk of heart attack or stroke due to blood clots.
Avoid low-oxygen environments: Avoid living at high altitudes, skiing, or climbing mountains. All reduce the oxygen levels in the blood even further.
Be good to your skin: To reduce itching, bathe in cool water, use a gentle cleanser and pat your skin dry. Adding starch, such as cornstarch, to the bath might help. Avoid hot tubs, heated whirlpools, and hot showers or baths.
Try not to scratch, as it can damage the skin and increase the risk of infection. Use lotion to keep the skin moist.
Avoid extreme temperatures: Poor blood flow increases the risk of injury from hot and cold temperatures. In cold weather, always wear warm clothing, particularly on the hands and feet. In hot weather, drink plenty of liquids and avoid exposure to sunlight.
Watch for sores: Poor circulation can make it difficult for sores to heal, particularly on your hands and feet. Inspect your feet regularly and tell your doctor about any sores.