What is Polycythemia vera?
Polycythemia vera (PV) is a rare type of blood cancer that causes an overproduction of red blood cells in the bone marrow. This results in the thickening of the blood, which can lead to an increased risk of blood clots, stroke, and heart attack.
PV is a rare disease, with an incidence rate of about 2 per 100,000 people per year. It usually affects people over the age of 60, but it can occur at any age. Men are more likely to develop the disease than women.
Symptoms
The symptoms of PV are often mild or absent in the early stages of the disease. However, as the disease progresses, symptoms may become more noticeable. The most common symptoms of PV include:
- Fatigue
- Headaches
- Dizziness
- Shortness of breath
- Itchy skin, especially after a warm bath or shower
- Redness, warmth, or swelling in the hands or feet
- Joint pain
- Increased sweating
- Enlarged spleen
Diagnosis
The diagnosis of PV is usually made through a series of tests, including a complete blood count (CBC), bone marrow biopsy, and genetic testing. A CBC will show an increase in the number of red blood cells, and possibly even the white blood cells, and platelets in the blood.
A bone marrow biopsy is used to confirm the diagnosis and to determine the extent of the disease. This is often done in an outpatient setting.
Genetic testing is used to look for mutations in the JAK2 gene, which is present in more than 95% of patients with PV.
Treatment
The goal of treatment for PV is to reduce the risk of blood clots and other complications. Treatment options include:
- Phlebotomy: This is the most common treatment for PV. It involves the removal of a certain amount of blood from the patient’s body, which reduces the number of red blood cells in the blood. The procedure is very similar to a blood donation.
- Medications: Medications, such as hydroxyurea or others, may be prescribed to reduce the number of red blood cells in the blood.
- Low-dose aspirin: This medication can help prevent blood clots.
- Blood thinners: Blood thinners, such as warfarin or heparin, may be prescribed to reduce the risk of blood clots.
- Radiation therapy: This treatment is used in some cases to reduce the size of an enlarged spleen.
- Bone marrow transplant: In rare cases, a bone marrow transplant may be recommended for patients with severe PV.