Definition

What is primary thrombocythemia?

Primary thrombocythemia is a blood clotting disorder that causes bone marrow to produce too many platelets. It’s also known as essential thrombocythemia.

Bone marrow is the spongelike tissue inside your bones. It contains cells that produce:

  • Red blood cells (RBCs), which carry oxygen and nutrients
  • White blood cells (WBCs), which help fight infections
  • Platelets, which enable blood clotting

A high platelet count can cause blood clots to develop spontaneously. Normally, your blood begins to clot to prevent a massive loss of blood after an injury. In people with primary thrombocythemia, however, blood clots can form suddenly and for no apparent reason.

Abnormal blood clotting can be dangerous. Blood clots may block the flow of blood to the brain, liver, heart, and other vital organs.

How common is primary thrombocythemia?

Primary thrombocythemia is rare. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of primary thrombocythemia?

Primary thrombocythemia may or may not cause symptoms. When symptoms occurs, they are due to the blockage of blood vessels by blood clots and may include:

  • Redness and warmth of the hands and feet, often with burning pain (erythromelalgia)
  • Tingling and other abnormal sensations in the fingertips, hands, and feet
  • Chest pain
  • Loss of vision or seeing spots
  • Headaches
  • Weakness
  • Dizziness
  • Bleeding, usually mild (such as nosebleeds, easy bruising, slight oozing from the gums, or bleeding in the digestive tract)

The spleen and liver may enlarge but usually not enough to cause symptoms.

Older people who may already have diseases that cause blood vessel damage, such as diabetes and high blood pressure, have a higher risk of complications caused by blood clots, such as transient ischemic attacks, stroke, and heart attack.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes primary thrombocythemia?

This condition occurs when your body produces too many platelets, which can lead to abnormal clotting. However, the exact cause for this is unknown. According to the MPN Research Foundation, approximately half of people with primary thrombocythemia have a gene mutation in the Janus kinase 2 (JAK2) gene. This gene is responsible for making a protein that promotes the growth and division of cells.

When your platelet count is too high due to a specific disease or condition, it’s called secondary or reactive thrombocytosis. Primary thrombocythemia is less common than secondary thrombocytosis. Another form of thrombocythemia, inherited thrombocythemia, is very rare.

Risk factors

What increases my risk for primary thrombocythemia?

There are many risk factors for primary thrombocythemia, such as:

  • Female gender
  • Age over 50

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is primary thrombocythemia diagnosed?

Your doctor will first perform a physical examination and ask you about your medical history. Make sure to mention any blood transfusions, infections, and medical procedures you’ve had in the past. Also tell your doctor about any prescription and over-the-counter (OTC) medications and supplements you’re taking.

If primary thrombocythemia is suspected, your doctor will run certain blood tests to confirm the diagnosis. Blood tests may include:

  • Complete blood count (CBC). A CBC measures the number of platelets in your blood.
  • Blood smear. A blood smear examines the condition of your platelets.
  • Genetic testing. This test will help determine whether you have an inherited condition that causes a high platelet count.

Other diagnostic testing may include bone marrow aspiration to examine your platelets under a microscope. This procedure involves taking a sample of bone marrow tissue in liquid form. It’s typically extracted from the breastbone or pelvis.

You’ll most likely receive a diagnosis of primary thrombocythemia if your doctor can’t find a cause for your high platelet count.

How is primary thrombocythemia treated?

Your treatment plan will depend on a number of factors, including your risk of developing blood clots.

You may not need treatment if you don’t have any symptoms or additional risk factors. Instead, your doctor may choose to carefully monitor your condition. Treatment may be recommended if you:

  • Are over age 60
  • Are a smoker
  • Have other medical conditions, such as diabetes or cardiovascular disease
  • Have a history of bleeding or blood clots

Treatment may include the following:

  • OTC low-dose aspirin (Bayer) may reduce blood clotting.
  • Prescription medications can lower the risk of clotting or reduce platelet production in the bone marrow.
  • Platelet pheresis. This procedure removes platelets directly from the blood.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage primary thrombocythemia?

The following lifestyles and home remedies might help you cope with primary thrombocythemia:

  • Controlling your blood pressure, cholesterol, and conditions such as diabetes can help reduce the risk of blood clots. You can do this by exercising regularly and eating a diet that largely consists of fruits, vegetables, whole grains, and lean protein.
  • It’s also important to quit smoking. Smoking increases your risk of blood clots.
  • Take all medications as prescribed.
  • Avoid OTC or cold medications that increase the risk of bleeding.
  • Avoid contact sports or activities that increase the risk of bleeding.
  • Promptly report abnormal bleeding or symptoms of blood clots to your doctor.
  • Before any dental or surgical procedures, make sure to tell your dentist or doctor about any medications you may be taking to lower your platelet count.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Review Date: August 26, 2018 | Last Modified: August 26, 2018

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