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Polycythemia Vera Symptoms, Causes, Diagnosis and Treatment

Polycythemia vera is a myeloproliferative disorder associated with a mutation in the enzyme Janus kinase-2 (JAK2) that causes neoplastic proliferation of hematopoietic progenitor cells. It causes high production of red blood cells as well as secondary production of white blood cells and platelets. Complications of the disease are associated with increased blood viscosity, including thrombosis. This activity reviews the assessment and treatment of polycythemia vera and highlights the role of the interprofessional team in improving the care of patients with this disease.

Polycythemia Vera

This disease gets worse slowly, usually over many years. It can be fatal if left untreated, but proper care can help you live a long life.

Most people with polycythemia vera aren’t 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.

What is Polycythemia Vera?

Polycythemia vera (PV) is a rare blood disorder characterized by an increase in all blood cells, especially red blood cells. The increased number of blood cells causes the blood to thicken and can lead to problems with blood flow. This can cause blood clots to form in the blood vessels, which can lead to strokes or damage to tissues and organs.

Symptoms of Polycythemia Vera:

At first, you may not notice any problems. When they do occur, symptoms of polycythemia vera may include:

  • Headaches
  • Double Vision
  • Dark or Blind Spots in Your Vision That Come and Go
  • Itching All Over Your Body, Especially After Being in Warm or Hot Water
  • Sweating, Especially at Night
  • Red Face That Looks Like Sunburn or a Rash
  • Weakness
  • Dizziness
  • Weight Loss
  • Difficulty Breathing
  • Hot or Burning Hands or Feet
  • Painful Inflammation of the Joints

you may also feel pressure or fullness under your ribs on the left side. This could mean that polycythemia vera has caused an enlarged spleen, an organ that helps filter blood.

What Causes Polycythemia Vera?

Polycythemia vera occurs in the bone marrow, the soft, spongy material in the center of bones. This is where new blood cells develop. Polycythemia vera begins when a single gene fails in a single stem cell in the bone marrow. In over 90% of cases, it is a gene called JAK2. The mutated gene tells the stem cell to continually reproduce. All of the reproduced cells continue to reproduce as well, until the abnormal cells outcompete the normal cells in the bone marrow. Most of the time, the JAK2 gene mutation is acquired, meaning it is not inherited from a family member. It happens for unknown reasons at some point in life. But there have been a few documented cases where multiple family members have developed polycythemia vera.

Diagnosis of Polycythemia Vera:

Your doctor will take a detailed medical history and do a physical exam.

Blood Tests:

If you have polycythemia vera, blood tests may show:

  • More red blood cells than normal and sometimes an increase in platelets or white blood cells
  • A higher percentage of red blood cells that make up the total blood volume (measurement of hematocrit)
  • High levels of the iron-rich protein in red blood cells that carry oxygen (hemoglobin)

Bone Marrow Aspiration or Biopsy:

If your doctor suspects that you have polycythemia vera, he or she may recommend that you have a sample of your bone marrow taken by bone marrow aspiration or biopsy.

A bone marrow biopsy is a sample of solid bone marrow material taken. A bone marrow aspiration is usually done at the same time. During an aspiration, your doctor takes a sample of the liquid part of your marrow.

Bone Marrow Examination:

In a bone marrow aspiration, a healthcare provider uses a thin needle to remove a small amount of liquid bone marrow, usually from a point at the back of the hip bone (pelvis). Often, a bone marrow biopsy is done at the same time. This second procedure removes a small piece of bone tissue and the bone marrow that contains it.

Specific Gene Testing:

If you have polycythemia vera, testing your bone marrow or blood can reveal the genetic mutation associated with the disease.

How is Polycythemia Vera Treated?

Treatment will depend on symptoms, age, and general health. It will also depend on the severity of the condition.

Treatment may include:

Phlebotomy. This procedure involves removing blood from the body. At first, this should be done frequently, such as once a week. Once enough blood has been removed to reduce the body’s iron stores (needed to produce blood quickly), it will not need to be done as often.

Certain medications, including chemotherapy. These medications help stop the bone marrow from making too many blood cells. They also keep blood flow and blood thickness close to normal.

Medications that may be used include:
  • Hydroxyurea to reduce the number of red blood cells produced by the bone marrow. This medication may be used when the number of other types of blood cells is also high.
  • Interferon to reduce blood counts.
  • Anagrelide to reduce platelet counts.
  • Ruxolitinib (Jakafi) to reduce red blood cell counts and reduce an enlarged spleen. This medication is used when hydroxyurea and other treatments have failed.
  • Taking aspirin to reduce the risk of blood clots may be an option for some people, but aspirin increases the risk of stomach bleeding.
  • Ultraviolet B light therapy may reduce the intense itching that some people experience.

Living with Polycythemia Vera:

There is no cure for polycythemia vera, but proper treatment can help reduce or delay any problems. Work with your doctor to create a treatment plan that is right for you. You should also be physically active to increase your heart rate and improve your circulation.

Other ways to improve your circulation include:
  • Stretching your legs and ankles
  • Avoiding extreme heat
  • Drinking plenty of water

This condition can lead to circulation problems, so take care of your hands and feet. Protect them from injury caused by cold and heat. For example, always wear shoes, even when you are at home. Wear gloves and warm socks in cold weather.

References

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