Polycythemia vera

What is Polycythemia vera?

Polycythemia vera is a rare blood disorder where your bone marrow produces too many red blood cells. Your bone marrow is the spongy tissue inside your bones that makes blood cells. When you have this condition, your body creates red blood cells much faster than normal.

These extra red blood cells make your blood thicker and harder to pump through your vessels. Thicker blood moves more slowly and can form clots more easily. This condition is a type of myeloproliferative neoplasm, which means it involves the abnormal growth of blood cells.

Polycythemia vera usually develops slowly over many years. Most people are diagnosed after age 60, though it can occur at any age. The condition requires ongoing monitoring and treatment to prevent serious complications like blood clots, strokes, or heart attacks.

Symptoms

  • Headaches and dizziness
  • Itching after a warm bath or shower
  • Reddish or purplish skin color, especially on the face
  • Fatigue and weakness
  • Blurred vision or blind spots
  • Ringing in the ears
  • Excessive sweating, especially at night
  • Shortness of breath
  • Numbness or tingling in hands and feet
  • Enlarged spleen causing fullness in the upper left abdomen
  • Unexplained weight loss
  • Easy bruising or bleeding

Some people have no symptoms early in the disease and discover the condition during routine blood work. Others experience vague symptoms like tiredness that can be mistaken for other conditions. Symptoms often develop gradually as red blood cell counts rise over time.

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Causes and risk factors

Polycythemia vera happens because of a genetic mutation in your bone marrow cells. More than 95 percent of people with this condition have a mutation in the JAK2 gene. This mutation is not inherited from your parents. It develops during your lifetime in the cells that produce blood. The mutation causes bone marrow cells to grow and divide without the normal controls that regulate blood cell production.

Risk factors include being over age 60, being male, and having a family history of the condition. Exposure to certain chemicals or radiation may increase risk, though this is not proven. Unlike many blood disorders, lifestyle factors like diet and exercise do not cause polycythemia vera. However, they do play an important role in managing complications once you have the condition.

How it's diagnosed

Polycythemia vera is diagnosed through blood tests that measure your red blood cell count and other markers. An elevated red blood cell count is the first clue. Men with RBC counts above 6.5 million cells per microliter and women above 6.0 million cells per microliter may have this condition. Your doctor will also check hemoglobin and hematocrit levels, which are typically high in polycythemia vera.

Rite Aid offers blood testing that includes red blood cell count measurement at Quest Diagnostics locations nationwide. Our panel can help identify elevated RBC levels that warrant further investigation. If your results show high red blood cell counts, your doctor may order genetic testing to look for the JAK2 mutation. They may also perform a bone marrow biopsy to examine how your marrow is producing blood cells.

Treatment options

  • Phlebotomy to remove excess blood and reduce red blood cell count, similar to donating blood
  • Low-dose aspirin to prevent blood clots
  • Hydroxyurea medication to slow bone marrow production of red blood cells
  • Interferon alfa injections for younger patients or those who cannot take hydroxyurea
  • Ruxolitinib for patients who do not respond to other treatments
  • Staying well hydrated to keep blood from getting too thick
  • Avoiding tobacco, which constricts blood vessels and increases clot risk
  • Regular exercise to improve circulation
  • Maintaining a healthy weight to reduce cardiovascular stress
  • Avoiding extreme temperatures, which can trigger symptoms

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Frequently asked questions

Many people with polycythemia vera live for 20 years or more after diagnosis with proper treatment. Life expectancy depends on how well the condition is managed and whether complications develop. Regular monitoring and treatment significantly improve outcomes. The main risks are blood clots, progression to other blood disorders, and cardiovascular problems.

There is currently no cure for polycythemia vera. However, treatment can effectively control the condition and prevent complications. Most people manage their symptoms well with regular phlebotomy and medication. The goal of treatment is to keep red blood cell counts in a safe range and reduce the risk of blood clots.

Most people with polycythemia vera need blood tests every few months to monitor their red blood cell count. Your doctor will check more frequently when starting treatment or changing medications. Regular monitoring helps ensure your blood counts stay in a healthy range. Testing frequency may decrease once your condition is well controlled.

Yes, polycythemia vera is classified as a blood cancer, specifically a myeloproliferative neoplasm. However, it grows very slowly and behaves differently from other cancers. Most people live for many years with proper management. In rare cases, it can progress to acute leukemia or myelofibrosis.

The itching, especially after warm baths, happens because of increased histamine release from blood cells. About 40 percent of people with polycythemia vera experience this symptom. The exact mechanism is not fully understood. Antihistamines and medications like interferon can help reduce itching in many cases.

While diet does not treat the underlying condition, healthy eating supports your overall health and reduces complications. Staying well hydrated helps keep blood less viscous. A diet rich in vegetables, fruits, and omega-3 fatty acids may reduce inflammation. Avoiding iron supplements is important because they can increase red blood cell production.

Polycythemia vera is a bone marrow disorder caused by a genetic mutation. Secondary polycythemia happens when something else causes your body to make more red blood cells, like lung disease or living at high altitude. The treatments differ because the underlying causes are different. Blood tests and genetic testing help doctors distinguish between them.

Many people need regular phlebotomy treatments for life to keep red blood cell counts safe. Some patients require phlebotomy every few weeks initially, then less often as their counts stabilize. Medications like hydroxyurea may reduce or eliminate the need for phlebotomy. Your treatment plan depends on how your body responds and your individual risk factors.

Yes, regular exercise is generally beneficial for people with polycythemia vera. Physical activity improves circulation and cardiovascular health. Talk to your doctor about appropriate exercise intensity, especially if you have symptoms like dizziness or chest pain. Stay well hydrated before, during, and after exercise.

Blood clots are the most dangerous complication, causing strokes, heart attacks, and deep vein thrombosis. Bleeding problems can also occur despite thick blood. About 10 to 20 percent of people eventually develop myelofibrosis, where scar tissue replaces bone marrow. A small percentage progress to acute leukemia, which is harder to treat.

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