Polycythemia Vera

What is Polycythemia Vera?

Polycythemia Vera is a slow-growing blood cancer where your bone marrow makes too many red blood cells. These extra cells cause your blood to become thicker than normal. Thicker blood moves more slowly through your veins and arteries, which can lead to serious problems like blood clots.

This condition is rare, affecting about 1 in every 100,000 people. It usually develops slowly over many years. Most people are diagnosed after age 60, though it can happen at any age. Polycythemia Vera is a lifelong condition, but with proper monitoring and treatment, most people live normal lives.

Your bone marrow is the soft tissue inside your bones where blood cells are made. In Polycythemia Vera, a genetic mutation causes your bone marrow to work overtime. It produces far more red blood cells than your body needs. This overproduction can also affect other blood cells like platelets and white blood cells.

Symptoms

  • Headaches and dizziness
  • Fatigue and weakness despite normal rest
  • Itching after warm showers or baths
  • Red or flushed face, especially on the cheeks
  • Blurred vision or blind spots
  • Ringing in the ears
  • Enlarged spleen causing left side abdominal pain
  • Burning sensation in hands or feet
  • Excessive sweating, especially at night
  • Bleeding or bruising easily

Many people with Polycythemia Vera have no symptoms at first. The condition is often discovered during routine blood work. As the disease progresses and blood becomes thicker, symptoms typically become more noticeable.

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Causes and risk factors

Polycythemia Vera is caused by a genetic mutation called JAK2 V617F. This mutation is found in about 95% of people with the condition. The mutation is not inherited from your parents. It happens randomly during your lifetime. The JAK2 mutation makes your bone marrow cells grow and divide out of control, producing too many red blood cells.

Scientists do not know exactly what triggers this mutation. Age is the biggest risk factor, with most cases occurring after age 60. Men are slightly more likely to develop the condition than women. Exposure to certain chemicals or radiation may increase risk, but this link is not fully proven. Having a family history of Polycythemia Vera or related blood disorders may slightly increase your risk.

How it's diagnosed

Polycythemia Vera is diagnosed through blood tests that measure your blood cell counts and check for genetic mutations. A complete blood count will show elevated red blood cells, and often high platelet counts and white blood cells too. Your doctor will also order a test for the JAK2 V617F mutation, which confirms the diagnosis in most cases. Serum viscosity testing measures how thick your blood has become.

Rite Aid offers blood testing that includes platelet count and basophil levels, which help detect and monitor Polycythemia Vera. Our testing is available at Quest Diagnostics locations nationwide. Early detection through regular blood work can catch this condition before serious complications develop. Your doctor may also perform a bone marrow biopsy to examine your bone marrow cells directly.

Treatment options

  • Phlebotomy treatments to remove excess blood, similar to donating blood
  • Low-dose aspirin to prevent blood clots
  • Medications like hydroxyurea to slow blood cell production
  • JAK inhibitors such as ruxolitinib for advanced cases
  • Drinking plenty of water to help thin your blood
  • Avoiding tobacco, which further thickens blood
  • Regular exercise to improve circulation
  • Managing stress, which can worsen symptoms
  • Avoiding extreme temperatures that trigger itching
  • Regular blood tests to monitor your condition

Concerned about Polycythemia Vera? Get tested at Rite Aid.

  • Simple blood draw at your nearest lab
  • Results in days, not weeks
  • Share results with your doctor
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Frequently asked questions

Polycythemia Vera is a blood cancer caused by a genetic mutation in your bone marrow. Regular polycythemia, also called secondary polycythemia, happens when your body makes extra red blood cells in response to low oxygen levels. Secondary polycythemia can be caused by lung disease, sleep apnea, or living at high altitude. Polycythemia Vera requires different treatment because it involves a mutation that keeps producing too many cells.

Polycythemia Vera cannot be cured, but it can be managed very effectively with treatment. Most people with this condition live normal lifespans with proper care. Treatment focuses on reducing your red blood cell count and preventing complications like blood clots. Regular monitoring and following your treatment plan are essential for staying healthy.

Phlebotomy frequency depends on how quickly your red blood cells increase. At first, you might need treatments every few days or weeks. Once your levels stabilize, you may only need treatment every few months. Your doctor uses blood tests to determine the right schedule. The goal is to keep your hematocrit level below 45% for most patients.

The itching happens because your body releases histamine when warm water touches your skin. People with Polycythemia Vera produce too many basophils, a type of white blood cell that releases histamine. This creates an intense, prickly itching sensation that can last 30 to 60 minutes. Taking cooler showers and using antihistamines can help reduce this symptom.

The thick blood in Polycythemia Vera can form dangerous blood clots in your veins or arteries. These clots can cause heart attacks, strokes, or clots in your legs or lungs. An enlarged spleen is common and can cause pain or rupture. Some people develop stomach ulcers or gout. In rare cases, Polycythemia Vera can progress to acute leukemia or myelofibrosis after many years.

Polycythemia Vera is not directly inherited from your parents. The JAK2 mutation that causes it happens during your lifetime, not at birth. However, having a family member with Polycythemia Vera or a related blood disorder does slightly increase your risk. If multiple family members are affected, genetic counseling may be helpful.

Diet cannot cure Polycythemia Vera, but healthy eating supports your overall treatment. Staying well hydrated helps keep your blood less thick. Eating foods low in iron may slow red blood cell production slightly. Avoiding excess alcohol reduces strain on your liver and spleen. A balanced diet with plenty of vegetables and lean protein supports your immune system during treatment.

Polycythemia Vera is a slow-growing condition that develops over many years. Some people have the mutation for years before symptoms appear. Once diagnosed, the disease progression varies from person to person. With treatment, most people maintain stable blood counts and good quality of life. Regular monitoring helps catch any changes early.

High altitudes can worsen Polycythemia Vera because lower oxygen levels trigger your body to make even more red blood cells. If you must travel to high altitudes, talk to your doctor first. They may adjust your treatment or recommend extra phlebotomy before your trip. Staying well hydrated at altitude is especially important for keeping your blood flowing properly.

Elevated red blood cell count and hemoglobin levels are the first signs. Men with hemoglobin above 16.5 and women above 16.0 may have Polycythemia Vera. Platelet counts above 400,000 per microliter are common. The JAK2 V617F mutation test is positive in about 95% of cases. High serum viscosity shows your blood has become too thick.

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