Polycythemia vera (PV) is a rare blood cancer that starts in the bone marrow, the soft center of the bones where new blood cells are produced. In people with PV, the bone marrow makes too many red blood cells, causing the blood to become thick and flow slowly. This increases the risk of blood clots, which can lead to serious complications like heart attacks or strokes.
PV is a slow-growing cancer that typically develops over many years. While it can be life-threatening if left untreated, proper care and management can help people with PV live long, fulfilling lives. The condition is most often diagnosed in people over 60 years old, but it can occur at any age and is more common in men than women.
In the early stages of PV, many people do not experience any symptoms. As the condition progresses, common signs and symptoms may include:
Headaches
Dizziness
Weakness
Itching (especially after warm showers or baths)
Sweating (particularly at night)
Blurred or double vision
Shortness of breath
Painful joint swelling
Redness or a feeling of fullness in the face
PV is caused by a mutation in the JAK2 or TET2 gene, which leads to the overproduction of red blood cells. In most cases, this genetic change occurs during a person's lifetime and is not inherited from parents. However, in rare instances, the faulty gene can be passed down from parent to child.
If not properly managed, PV can lead to various complications, such as:
Blood clots (which may cause heart attacks, strokes, or deep vein thrombosis)
Enlarged spleen or liver
Chest pain or heart failure (due to reduced blood flow to organs)
Stomach ulcers
Gout
Kidney stones
In some cases, PV can progress to more serious blood disorders, such as acute leukemia or myelofibrosis.
To diagnose PV, your doctor will perform a physical exam and ask about your symptoms. They may also order blood tests, such as:
Complete blood count (CBC) to measure the number of red blood cells, white blood cells, and platelets
Blood smear to check for other diseases sometimes linked to PV
EPO level test to measure the amount of erythropoietin (a hormone that stimulates red blood cell production) in your blood
In some cases, a bone marrow biopsy may be necessary to confirm the diagnosis. This procedure involves removing a small sample of bone marrow, usually from the hip bone, for examination under a microscope.
Treatment for PV aims to reduce the number of red blood cells in the body and prevent complications. The most common treatments include:
Phlebotomy (blood removal) to thin the blood and improve circulation
Low-dose aspirin to reduce the risk of blood clots
Medications such as hydroxyurea, interferon alfa, or ruxolitinib to lower blood cell production
Antihistamines to relieve persistent itching
In addition to medical treatment, lifestyle changes can help manage PV symptoms and reduce the risk of complications. These include not smoking, engaging in light exercise, and taking cool showers or baths to alleviate itching.
While there is no cure for polycythemia vera, proper treatment and management can help people with this condition live long, healthy lives. If you have been diagnosed with PV, it's essential to work closely with your healthcare team and seek support from loved ones or support groups when needed.
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