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Polycythemia Vera

Polycythemia Vera (PV) is a disorder that stimulates an overproduction of red blood cells, white blood cells, and platelets. Some patients with PV will not demonstrate any symptoms at all, but many will experience easy bruising or bleeding with little or no injury. Also, the blood may be thick and gummy, causing it to clot in tiny blood vessels. If clotting occurs in the tiny blood vessels of the fingers and toes, a patient may experience numbness or burning. If it occurs in the brain, it may lead to mental confusion or, in more serious cases, a stroke.

Occasionally, the increased blood volume also causes congestion and enlargement of the spleen or liver. These organs may become enlarged because they are trying to produce extra blood cells, a process called extramedullary hematopoiesis. If the spleen becomes too enlarged, it can cause discomfort after eating, difficulty breathing, and chest pain.

Every year, there are five new cases of PV per 1,000,000 people. The average age of a person diagnosed with PV is 60 years old, although it can affect both young and old people, and it occurs more often in males than females. There is no known cure.

Diagnostic Tests

To diagnose PV, your doctor will order a blood test to count the number of cells in your blood. He or she may also perform a blood smear to determine if the cells appear normal. If these tests are abnormal, you may be referred to a hematologist, a doctor who specializes in diagnosing and treating bone marrow and blood disorders. Further special testing will likely be recommended.

Treatment Overview

The primary goal of treatment is to lower the number of cells in your blood. The treatment you receive will depend on your age, health, if you are having any symptoms, and the results of your blood test. It may include:

Phlebotomy is the process of removing blood from the body to reduce cell levels. It can be repeated when necessary.

Chemotherapy

Chemotherapy uses medication to slow the production of blood cells in the body. One type of medication often used to treat PV is hydroxyurea.

Biological Therapy

Biological Therapy, or immunotherapy, uses substances made naturally in the body to stimulate or bolster your own immune response to the disorder. One type of natural substance used to treat PV is alpha interferon.

Radioactive Drugs

A Radioactive drug called P32, or radioactive phosphorous, is sometimes given to patients with PV. Once injected, the medication travels to the bone marrow and destroys the overactive marrow cells. This treatment affects the white blood cells and platelets quickly, but may take about three months before affecting red blood cells. Because of this, your doctor may order phlebotomy treatments for several weeks after you receive P32.

Drug Treatments

Your doctor might prescribe additional medication to combat the symptoms of PV. To control itching, you may be given cholestyramine, cyproheptadine, cimetidine, or psoralen plus ultraviolet light treatment. You might also be given medication to counter the symptoms of high uric acid levels in the blood or swelling in the toes. Your doctor is the best person to tell you which treatment is best for you.

Managing Polycythemia Vera

In addition to the treatment recommended by your doctor, there are steps you can take to help reduce or prevent the symptoms of PV:

Stop chewing or smoking tobacco. Your doctor or nurse can recommend programs in your community to help you do this.
Balance activities with regular rest periods. This ensures that your body conserves energy.
Avoid sodium or salt rich foods. These foods may cause fluid retention and make some symptoms worse.
Engage in light exercise, such as walking. This can be helpful in promoting circulation and the function of your heart.
Breathe deeply and cough periodically. Deep breaths and coughing can help keep your airways open and prevent infection.
Bathe or shower in cool water if warm baths cause itching.
Dry your skin gently after bathing.
Avoid scratching the skin.
Use lotion to maintain skin moisture.
Protect your hands and feet from injury, heat, cold and pressure.
Avoid straining when having bowel movements.
Eat a well balanced diet to maintain proper weight.
Perform leg and ankle exercises to prevent clots from forming in the deep veins of the legs. Your doctor or nurse will show you these exercises.
Inspect your feet regularly and report any sores to your doctor.

It is important that you be alert for any change or increase in symptoms. If this occurs, it may mean that you need additional or different treatments, and you should contact your doctor right away. The following symptoms require prompt attention:

Enlargement of the Spleen Symptoms

discomfort or bloating in the abdomen
enlargement of the abdomen
weight loss
backache
stomach pain
blood in your stools or rectal bleeding

Clotting Symptoms

redness or burning sensation in the fingers, hands, and feet
fatigue
dizziness
headaches
visual changes
confusion in speech
numbness, tingling, or weakness in arms or legs
pain or swelling in arms or legs
shortness of breath
ruddy color in the face, hands, and feet
chest pain
sweating
increased cough
coughing up blood

Cardiopulmonary Problem Symptoms

dizziness
headaches
pressure in the head
shortness of breath
weakness and fatigue
pain in hands or feet
visual disturbances

Irritated Skin Symptoms

itching, especially after bathing in hot water or sleeping in bed
rash

Follow-Up Care and Prognosis

PV requires regular appointments with your doctor. He or she will want to discuss your symptoms and do periodic blood counts to evaluate your response to treatment.

Polycythemia Vera is a disorder that cannot be cured, but it can be controlled with treatment. Some patients may only need minimal care and observation, while others may require more intensive treatment. In a small number of patients, PV may transform into another type of disorder, especially myelofibrosis or acute leukemia.

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