What is Essential thrombocythemia (ET)?

Essential thrombocythemia (ET) is a type of blood cancer. ET can result from a mutation in blood-forming stem cells, which causes blood cells (particularly platelets) to produce uncontrollably. However, research is still being done to understand the additional causes of ET. ET is one of three myeloproliferative neoplasms (MPNs).

Signs and Symptoms of ET

Most people with ET show no signs or symptoms. However, if signs and symptoms occur they may include:

  • Headache
  • Throbbing or burning pain in feet or hands
  • Lightheadedness or Dizziness
  • Enlarged spleen
  • Thrombosis (Abnormal clotting)
  • Bleeding (less common)
  • Fatigue
  • Weakness
  • Sweating

Diagnosing ET

Blood tests such as a “complete blood count” (CBC) are used to measure blood cells and platelets. Normal platelet levels are between 150,000 to 400,000 platelets per microliter (μL) of blood. If a patient’s platelet level is above 600,000/μL and remains high over time, ET is considered. However, your doctor may suggest further tests to rule any underlying cause. 

Additional tests may include:
  • Testing for the presence of JAK2 mutation or other genetic mutations
  • Bone marrow biopsy and aspirate, helps to rule out other causes and confirm an ET diagnosis

Treatment of ET

There may be several health care providers involved in your care including a hematologist/oncologist, nurse, nurse navigator, oncology social worker and pharmacist. Each of these professionals can help guide you to information and resources that may help you both during and after your treatment. If you do not meet these professionals upfront, ask your doctor how you might arrange an appointment with them. In addition, you may want to contact your insurance carrier to ask for a case manager to be assigned to you to assist in managing your treatment coverage options. 

There are several approaches to treatment depending on your Essential Thrombocythemia diagnosis and its progression. There are various drugs used in the treatment of ET, and many of them have very different approaches to slowing the disease process and/or controlling the effects of the disease. With any drugs, side effects will likely exist; your doctor or nurse should review these with you before starting. Options may include: 

  • Watch & Wait, is when patients who are symptom and sign free and exhibit slow disease progression are not treated at the time of diagnosis. Some people remain stable and symptom free for many years. However, it is very important that individuals be closely monitored through regular medical checkups and examinations to detect signs or symptoms of disease progression. 
  • Chemotherapeutic agents, which may reduce the platelet count, usually given by mouth (orally). Some agents may be given intravenously (into the vein). 
  • Aspirin, taken orally, in low doses. Can help reduce the risk of clotting. 
  • Biological therapies, which may also help reduce platelet counts, and is usually given by injection. 
  • Clinical Trials. Because doctors are still trying to find more effective treatments for ET, clinical trials are an important option to consider. Clinical trials are research studies to test promising new drugs or treatment approaches. Participating in a clinical trial may give you the opportunity to be among the first to benefit from the most advanced treatment available. If you are interested in a clinical trial, ask your healthcare team about studies that you might qualify for. More information on clinical trials and how to participate in one. 

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