Polycythemia vera

Polycythemia vera also called primary polycythemia — is rare and usually develops slowly  blood disorder in which your bone marrow makes too many red blood cells. Polycythemia vera also may result in production of too many of the other types of blood cells — white blood cells and platelets. But it’s the excess red blood cells that thicken your blood and cause most of the concerns associated with polycythemia vera.   You may have it for years without noticing signs or symptoms. Often, polycythemia vera is found during a blood test done for some other reason. Without treatment, polycythemia vera can be life-threatening.

Polycythemia vera symptoms

In its early stages, polycythemia vera usually doesn’t cause any signs or symptoms. However, as the disease progresses, you may experience:

  • Headache
  • Dizziness
  • Itchiness, especially following a warm bath or shower
  • Redness of your skin
  • Shortness of breath
  • Breathing difficulty when you lie down
  • Numbness, tingling, burning or weakness in your hands, feet, arms or legs
  • Chest pain
  • A feeling of fullness or bloating in your left upper abdomen due to an enlarged spleen
  • Fatigue

Because polycythemia vera causes your blood to thicken and slows blood flow, it increases your risk of developing blood clots. If a blood clot occurs in your head, it can cause a stroke. Seek emergency medical care if you have any of the following signs or symptoms of a stroke:

  • Sudden numbness, weakness, or paralysis of your face, arm or leg — usually on one side of your body
  • Sudden difficulty speaking or understanding speech (aphasia)
  • Sudden blurred, double or decreased vision
  • Sudden dizziness, loss of balance or loss of coordination
  • A sudden, severe headache or an unusual headache, which may be accompanied by a stiff neck, facial pain, pain between your eyes, vomiting or altered consciousness
  • Confusion, or problems with memory, spatial orientation or perception

Polycythemia vera causes

The problem with blood cell production associated with polycythemia vera is caused by a change, or mutation, to DNA in a single cell in your bone marrow. In polycythemia vera, researchers have found this mutation to be a change in a protein switch that tells the cells to grow. Specifically, it’s a mutation in the protein JAK2 (the JAK2 V617F mutation). More than 90 percent of people with polycythemia vera, and about half the people with other myeloproliferative disorders, have this mutation. Doctors and researchers don’t yet understand the full role of this mutation and its implications for treating the disease.

The DNA mutation that causes polycythemia vera occurs after conception — meaning that your mother and father don’t have it — so it’s acquired, rather than inherited from a parent. Researchers and doctors don’t know what causes the mutation.

Polycythemia vera diagnosis

Doctors most frequently use blood tests to diagnose polycythemia vera. In fact, in many cases, polycythemia vera is discovered through blood tests performed for other reasons. If you have polycythemia vera, blood tests may reveal:

An increase in the number of red blood cells and, in some cases, an increase in platelets or white blood cells.
Elevated hematocrit measurement, the percentage of red blood cells that make up total blood volume.
Elevated levels of hemoglobin, the iron-rich protein in red blood cells that carries oxygen.

Lower than normal levels of oxygen in your blood.
Very low levels of erythropoietin (EPO), a hormone that stimulates bone marrow to produce new red blood cells; the excess red blood cells turn off the production of EPO by your kidneys.

Polycythemia vera treatment

Polycythemia vera is a chronic condition that can’t be cured. Treatment focuses on reducing your amount of blood cells. In many cases, treatment can prevent complications from polycythemia vera and decrease or eliminate the disease’s signs and symptoms.

Treatment may include:

Phlebotomy. This is usually the first treatment option for most people with polycythemia vera. Phlebotomy involves drawing a certain amount of blood from one of your veins. This reduces the number of blood cells and decreases your blood volume, making it easier for your blood to function properly. How often you need phlebotomy depends on the severity of your condition.
Medication to decrease blood cells. Phlebotomy alone may not be enough to control the signs, symptoms and complications of polycythemia vera in some people. If this is the case for you, your doctor may prescribe medications, such as hydroxyurea or anagrelide, to suppress your bone marrow’s ability to produce blood cells. Radioactive phosphorus (phosphorus 32 or p-32) also can be used to reduce the number of red blood cells. Interferon-alpha may be used to stimulate your immune system to fight the overproduction of red blood cells.
Low-dose aspirin. Your doctor may recommend that you take a low dose of aspirin to reduce your risk of blood clots. Low-dose aspirin may also help reduce burning pain in your feet or hands.
Therapy to reduce itching. If you have bothersome itching, your doctor may prescribe medication, such as antihistamines or H-2-receptor blockers, or recommend ultraviolet light treatment to relieve your discomfort.

Lifestyle with polycythemia vera

If you have polycythemia vera, be sure to:

  • Exercise. Moderate exercise, such as walking, can improve your blood flow, which decreases your risk of blood clots. Leg and ankle stretches and exercises also can improve your blood circulation.
  • Avoid tobacco. Using tobacco can cause your blood vessels to narrow, increasing the risk of heart attack or stroke due to blood clots.
  • Be good to your skin. To reduce itching, bathe in cool water and pat your skin dry. Avoid hot tubs, heated whirlpools, and hot showers or baths. Try not to scratch, as it can damage your skin and increase the risk of infection. Use lotion to keep your skin moisturized.
  • Avoid extreme temperatures. Poor blood flow increases your risk of injury from hot and cold temperatures. In cold weather, always wear warm clothing, particularly on your hands and feet. In hot weather, protect yourself from the sun and drink plenty of liquids.
  • Watch for sores. Poor circulation can make it difficult for sores to heal, particularly on your hands and feet. Inspect your feet regularly and tell your doctor about any sores.