Essential Thrombocytosis (Primary Thrombocythemia)

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Essential Thrombocytosis (Primary Thrombocythemia)


Essential Thrombocytosis, also known as Essential Thrombocythemia is myeloproliferative neoplasm (a blood cancer that occurs when the body makes too many white, or red bood cells, or platelets) that develops when the bone marrow doesn’t work normally as it makes too many platelets increasing the risk of blood clots and bleeding.

Essential Thrombocytosis, usually develops around 60 years of age, but it can also develop in younger people, especially women of child-bearing age.

In rare cases, Essential Thrombocytosis develops into Polycythemia Vera, Myelofibrosis, Myelodysplastic Syndromes (MDS) or Acute Myelogenous Leukaemia (AML).


Essential Thrombocytosis might not cause any symptoms, but when present, they are often related to clot formation or bleeding issues. One should contact the doctor if you have:

  • pain, redness, swelling and warmth in the feet and legs
  • headaches
  • dizziness
  • weakness
  • vision changes such as blurred vision
  • easy bruising or bleeding
  • bleeding from the gastrointestinal tract, skin, eyes, gums or urinary tract
  • abdominal discomfort or feeling of fullness if the spleen or liver is enlarged
  • tingling in the hands and feet
  • sweating
  • itchiness
  • slightly higher than normal temperature

Essential Thrombocytosis is usually suspected when the doctor sees a problem with the blood during a routine blood test. The doctor then asks about the symptoms and will do a physical exam to check if the spleen or liver is enlarged. Based on this information, the doctor will order tests to check for Essential Thrombocytosis or other health problems These tests include:

  • Complete Blood Count (CBC) to measure the number and quality of white blood cells, red blood cells and platelets.
  • Blood Chemistry Tests to show how well certain organs are working
  • Reverse Transciptase Polymerase Chain Reaction (RT-PCR) to see if the cells in a sample of blood or bone marrow have the JAK2 mutation.
  • Bone Marrow Aspiration and Biopsy to confirm whether or not one has Essential Thrombocytosis.

The treatment for Essential Thrombocytosis is based on the risk of developing complications, such as bleeding and blood clots. Patient are usually split up into low, intermediate and high risk of bleeding/ blood clotting groups (based on their age, their medical history, their blood counts and their lifestyles).

Patients are considered low risk if they don’t have any signs or symptoms of Essential Thrombocytosis other than a higher than normal platelet count and if they are under 40 years of age. Most low risk patients, especially those with no other cardiovascular risk factors, will not need treatment unless complications develop.

Patients are intermediate risk if they are between 40 and 60 years of age and if they have some symptoms that don’t have high-risk features. People who are at intermediate risk may not need treatment unless complications develop.

Patients with high risk need immediate treatment. People are considered high-risk if they are older than 60 years of age and they have of these high-risk features:

  • they have had blood clots or bleeding issues in the past
  • they have high blood pressure or diabetes
  • they smoke or are obese
  • they have a very high platelet count

Drug Therapy:
Speak to your doctor about the various drugs that could be used for treatment.

Plateletpheresis is a procedure that removes platelets from the blood. It can temporarily lower the platelet count. It is only used when the platelet count is extremely high and needs to be lowered quickly to treat clotting complications.