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Essential Thrombocythemia

Essential Thrombocythemia (ET), or primary thrombocythemia, is a disorder that causes the overproduction, and eventual failure, of platelets in the blood. About one-third of patients with ET will demonstrate no symptoms at all, but many will experience easy bruising or bleeding with little or no injury. Patients may also experience an enlarged spleen, and some will demonstrate thick, slow-moving blood that has a tendency to clot. If clotting occurs in the tiny blood vessels of the fingers and toes, there may be numbness or burning. If it occurs in the brain, there may be mental confusion or, in more serious cases, a stroke.

Every year, there is one new case of ET per 100,000 people. It usually occurs in adults between the ages of 50 to 70, although it can occur in both young and old people, and it affects both males and females equally. There is no known cure.

Diagnostic Tests

To diagnose ET, your doctor will order a blood test to count the number of cells in your blood. If the results are abnormal, you may be referred to a hematologist, a doctor who specializes in diagnosing and treating bone marrow and blood disorders.

Treatment Overview

The primary goal of treatment is to lower the number of platelets 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:

Monitoring
Plateletpheresis
Chemotherapy
Biological therapy
Other Medication

Monitoring

In some patients, the best treatment is to monitor the disorder for any increase in symptoms and have a doctor do periodic blood counts. This may be the only management ever needed.

Plateletpheresis

During plateletpheresis, a special machine filters the excess platelets out of the blood. This is usually done only when the platelet count is very high, and the effect is temporary. Chemotherapy

Chemotherapy uses medication to lower the number of platelets in the blood. Medications used to treat ET include hydroxyurea and anagrelide. Your doctor will determine which medication is best for you. Biological Therapy

Biological therapy, or immunotherapy, uses substances made naturally in the body to bolster your own immune response. Alpha-interferon is a natural substance often used to treat ET. Other

Aspirin is sometimes prescribed by a physician to relieve discomfort at the tips of fingers and toes.

Managing Essential Thrombocythemia

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

Avoid bumping or bruising yourself.
Use an electric razor when shaving and be cautious when using nail trimmers, knives, etc.
Wear hard-soled shoes, gloves, and long pants when working outside (i.e. gardening).
Use a sponge toothbrush if you have problems with gum bleeding. Your doctor or nurse can tell you if you need to use one and where it can be acquired.
Avoid aspirin or aspirin-like medications (for example, Motrin, ibuprofen, or other anti-inflammatory drugs) unless your doctor has told you otherwise. These medications can affect blood clotting. Also, be sure to inform your doctor of all over-the-counter medications that you take (including vitamins, herbs and dietary supplements).
Engage in light exercise, such as walking, to stimulate circulation and improve energy levels.
Perform leg and ankle exercises to prevent clots from forming in the deep veins of your legs. Your doctor or nurse will show you these exercises.

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:

Bleeding Symptoms

easy bruising
bleeding for no apparent reason
unusually heavy or prolonged bleeding
nose or gum bleeding
bleeding of mucous membrane
petechiae, or tiny areas of pinpoint bleeding on the skin of the arms or legs
black or bloody stools
blood in the urine

Clotting Symptoms

redness or burning sensations in the fingers, hands, and feet
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
chest pain
increased coughing (possibly with blood)

Follow-Up Care and Prognosis

ET 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.

Prognosis depends upon the symptoms you may have, your age, and the treatment you receive. Some patients may only need minimal care and observation, while other patients may require more intensive treatment. In a very small percent of patients, ET may evolve into other chronic disorders or transform into acute leukemia.

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