Learn About Essential Thrombocythemia

What is the definition of Essential Thrombocythemia?

Essential thrombocythemia (ET) is a condition in which the bone marrow produces too many platelets. Platelets are particles in the blood that aid in blood clotting.

What are the alternative names for Essential Thrombocythemia?

Primary thrombocythemia; Essential thrombocytosis

What are the causes of Essential Thrombocythemia?

ET results in an overproduction of platelets. The higher number of platelets may increase the risk of clotting. However, bleeding may occur with very high platelet count because blood clots use up the body's platelets. Untreated, ET worsens over time.

ET is part of a group of conditions known as myeloproliferative disorders. Others include:

  • Chronic myelogenous leukemia (an overproduction of white blood cells that starts in the bone marrow)
  • Polycythemia vera (bone marrow disease that leads to an abnormal increase in the number of red blood cells)
  • Primary myelofibrosis (disorder of the bone marrow in which the marrow is replaced by fibrous scar tissue)

Many people with ET have a variation of a gene (JAK2, CALR, or MPL).

ET is most common in middle- to older-age people. It can also sometimes be seen in younger people, especially women.

What are the symptoms of Essential Thrombocythemia?

Symptoms may include any of the following:

  • Headache
  • Tingling, coldness, or blueness in the hands and feet
  • Feeling dizzy or lightheaded
  • Vision problems
  • Mini-strokes (transient ischemic attacks) or stroke

If bleeding is a problem, symptoms may include any of the following:

  • Easy bruising and nosebleeds
  • Bleeding from the gastrointestinal tract, respiratory system, urinary tract, or skin
  • Bleeding from the gums
  • Prolonged bleeding from surgical procedures or tooth removal
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What are the current treatments for Essential Thrombocythemia?

If you have life-threatening complications, you may have a treatment called platelet pheresis. It quickly reduces the number of platelets in the blood.

Long-term, medicines are used to decrease the platelet count to avoid complications. The most common medicines used include hydroxyurea, interferon-alpha, or anagrelide.

Aspirin at a low dose (81 to 100 mg once or twice per day) may decrease clotting episodes.

Many people do not need any treatment, but they must be followed closely by their health care provider.

Who are the top Essential Thrombocythemia Local Doctors?
Mario Cazzola
Elite in Essential Thrombocythemia
Elite in Essential Thrombocythemia
Rome, IT 

Mario Cazzola practices in Rome, Italy. Mr. Cazzola and is rated as an Elite expert by MediFind in the treatment of Essential Thrombocythemia. His top areas of expertise are Chronic Obstructive Pulmonary Disease (COPD), Essential Thrombocythemia, Myeloproliferative Neoplasms (MPN), Bone Marrow Transplant, and Splenectomy.

Martin Griesshammer
Elite in Essential Thrombocythemia
Elite in Essential Thrombocythemia
Bochum, NW, DE 

Martin Griesshammer practices in Bochum, Germany. Mr. Griesshammer and is rated as an Elite expert by MediFind in the treatment of Essential Thrombocythemia. His top areas of expertise are Polycythemia Vera, Myeloproliferative Neoplasms (MPN), Essential Thrombocythemia, and Splenomegaly.

 
 
 
 
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Hagop M. Kantarjian
Elite in Essential Thrombocythemia
Oncology | Hematology
Elite in Essential Thrombocythemia
Oncology | Hematology

MD Anderson

2130 W Holcombe Blvd, Univ Of Tx M D Ande Life Sciences Pla, 
Houston, TX 
Languages Spoken:
English

Hagop Kantarjian is an Oncologist and a Hematologist in Houston, Texas. Dr. Kantarjian and is rated as an Elite provider by MediFind in the treatment of Essential Thrombocythemia. His top areas of expertise are Chronic Myelogenous Leukemia (CML), Acute Myeloid Leukemia (AML), Acute Lymphoblastic Leukemia (ALL), Bone Marrow Transplant, and Bone Marrow Aspiration.

What is the outlook (prognosis) for Essential Thrombocythemia?

Outcomes may vary. Most people can go for long periods without complications and have a normal lifespan. In a small number of people, complications from bleeding and blood clots can cause serious problems.

In rare cases, the disease can change into acute leukemia or myelofibrosis.

What are the possible complications of Essential Thrombocythemia?

Complications may include:

  • Acute leukemia or myelofibrosis
  • Severe bleeding (hemorrhage)
  • Stroke, heart attack, or blood clots
When should I contact a medical professional for Essential Thrombocythemia?

Contact your provider if:

  • You have unexplained bleeding that continues longer than it should.
  • You notice chest pain, leg pain, confusion, weakness, numbness, or other new symptoms.
What are the latest Essential Thrombocythemia Clinical Trials?
A Randomized Phase 2 Trial of ASTX727 +/- Iadademstat in Accelerated/Blast-Phase Philadelphia Chromosome-Negative Myeloproliferative Neoplasms (MPNs)

Summary: This phase II trial compares the effect of ASTX727 in combination with iadademstat to ASTX727 alone in treating patients with accelerated or blast phase Philadelphia chromosome negative myeloproliferative neoplasms (MPNs). ASTX727 is a combination of two drugs, cedazuridine and decitabine. Cedazuridine is in a class of medications called cytidine deaminase inhibitors. It prevents the breakdown of ...

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A Multicenter, Open-Label, Extension Study Evaluating the Safety and Efficacy of Bomedemstat for the Treatment of Participants Enrolled in a Prior Bomedemstat Clinical Study

Summary: The primary purpose of the study is to transition participants into an extension study to collect long-term safety and efficacy data. The study will include participants who are safely tolerating bomedemstat, receiving clinical benefit from its use in estimation of the investigator, and have shown the following criteria: * Participants from the IMG-7289-202/MK-3543-005 (NCT05223920) study must hav...

Who are the sources who wrote this article ?

Published Date: February 03, 2025
Published By: Warren Brenner, MD, Oncologist, Lynn Cancer Institute, Boca Raton, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Gotlib J. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 152.

Marcellino BK, Mascarenhas J, Iancu-Rubin C, Kremyanskaya M, Najfeld V, Hoffman R. Essential thrombocythemia. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 71.

National Cancer Institute website. Myeloproliferative neoplasms treatment (PDQ) -- health professional version. www.cancer.gov/types/myeloproliferative/hp/myeloproliferative-neoplasms-treatment. Updated September 27, 2024. Accessed February 11, 2025.