Polycythemia Vera

What is polycythemia vera?

Polycythemia vera is a blood disorder in which there is an increase in all blood cells, particularly red blood cells. The increase in blood cells makes the blood more viscous (thick), leading to strokes or tissue and organ damage.

Polycythemia vera occurs in approximately one in 100,000 people.

What causes polycythemia vera?

Polycythemia vera is one type of myeloproliferative disorder (MPD). Bone marrow contains stem cells that have the ability to mature into adult blood cells that enter the circulation, including red blood cells, white blood cells, and platelets. These stem cells are referred to as pluripotent hematopoeitic precursor cells, or PHPC, due to their versatility. Each PHPC can reproduce itself (clone), in addition to producing daughter cells, called blasts.

In MPD, one abnormal PHPC clone has an ability to overgrow and self-renew more effectively then normal PHPC clones. In polycythemia vera, there may be an increase in just the red blood cells, both the red and white blood cells, or in the red and white blood cells and platelets.

The cause of the abnormal PHPC clone is unknown.

What are the symptoms of polycythemia vera?

When there is an increase blood volume and viscosity (thickness), complications associated with this disease can occur. The following are the most common symptoms of polycythemia vera. However, each individual may experience symptoms differently. Symptoms may include:

• fatigue
• headache
• dizziness
• shortness of breath
• visual disturbances
• inability to concentrate
• night sweats
• flushed complexion
• nosebleeds
• bleeding gums
• excessive menstrual bleeding
• hemoptysis (coughing up blood)
• bruising
• itchy skin (particularly after a hot bath)
• gout
• numbness
• high blood pressure (hypertension)

Arterial or venous thrombosis can occur, resulting in a heart attack, stroke, or pulmonary embolism. The symptoms of polycythemia vera may resemble other blood disorders or medical problems. Always consult your physician for a diagnosis.

How is polycythemia vera diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for polycythemia may include additional blood tests to observe the increased number of red blood cells in the body, and distinguish it from other conditions which could cause the red blood cell count to increase (such as with certain cardiac and respiratory diseases, erythremia, and certain tumors).

Treatment for polycythemia vera:

Specific treatment for polycythemia vera will be determined by your physician based on:

• your age, overall health, and medical history
• extent of the disease
• your tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

Treatment may include:

• phlebotomy - a procedure that involves removing blood from the body.
• certain medications, including chemotherapy
• bone marrow transplantation