Essential thrombocythemia (ET)


What is it about ?

A platelet (thrombocyte) is a blood cell that plays a role in blood clotting. When bleeding, several platelets clump together and form a clot that stops the bleeding. Each cubic millimeter (mm3) blood normally contains between 150,000 and 360,000 platelets. Platelets form in the bone marrow.

Essential thrombocythemia (ET) is a progressive and chronic malignant disease of the blood (myeloproliferative disease) characterized by an overgrowth of platelets in the bone marrow. So there is too many platelets in the blood (thrombocytosis). The condition is sometimes hereditary.

In whom and how often does it occur?

Each year, there are 6 to 25 new cases of essential thrombocythemia per million population. Usually, the condition is more common in older people, especially after the age of 50 to 60 years.

How to recognize it?

Since there are too many platelets, the blood tends to clot too quickly and therefore to form a clot (thrombus). This clot can block a blood vessel, a phenomenon known as thrombosis. This can happen in an artery as well as in a vein and increase the risk of heart attack (myocardial infarction), ofcerebral infarction and of thrombosis of the blood vessels in the legs (deep vein thrombosis).

Despite this increased risk, there may be no symptoms or complaints. Often, essential thrombocythemia is discovered by chance with a routine blood test.

If there are any complaints, it is often severe pain in the feet or fingers. This pain is caused by a lack of oxygen in the tiny blood vessels in the feet and fingers.

In pregnant women, the presence of essential thrombocythemia (ET) is synonymous with increased risks for both mother and child. The miscarriages are much more common in women with ET than in healthy women.

Because not all platelets are of good quality, bleeding inside the body (internal bleeding) can also occur more quickly.

How is the diagnosis made?

The doctor will first check whether the excess platelets are not a temporary reaction of the body to a stressful situation, such as a serious infection, bleeding, a disease that affects the whole body (systemic disease) or a inflammatory disease, intense exertion, tissue damage, surgery, …

If the excess platelets is not due to temporary reversible factors and persists, the doctor will refer you to a doctor specializing in blood diseases (hematologist) for additional examinations. The diagnosis is made using four criteria, based on blood tests, bone marrow tests and genetic tests.

What can you do ?

Try tostop smoking, because tobacco further increases the risk of thrombosis.

What can your doctor do?

The treatment is based on 2 pillars: reducing the risk of clots and reducing the number of platelets. The main goal of treatment is to prevent clots from forming with acetylsalicylic acid (aspirin). This medicine makes the platelets less ‘sticky’ and thus reduces the risk of clotting. This is often the only treatment.

People at high risk for thrombosis also need medicines that slow down the production of platelets. However, these drugs can have considerable side effects, and some of them even promote the progression of the disease to a form of leukemia.

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Source

Foreign clinical practice guide ‘Essential thrombocythemia (ET)’ (2000), updated on 04.05.2017 and adapted to the Belgian context on 19.10.2019 – ebpracticenet