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Diagnosis and Treatment of ET

Diagnosis

Diagnosis of essential thrombocythemia requires blood testing. A complete blood count measures blood cells and platelets. Normal platelet levels are between 150,000 to 400,000 platelets per microliter (μL) of blood. Consistently elevated platelet levels may be a sign of essential thrombocythemia. Your doctor may order the following tests to gather more information or rule out other causes:

  • Testing for the presence of JAK2 mutation or other genetic mutations (e.g. CALR, MPL)
  • Bone marrow biopsy and aspirate, helps to rule out other causes and confirm an ET diagnosis

Treatment

Different health care providers involved in your care may include a hematologist/oncologist, a nurse, a nurse navigator, an oncology social worker or a pharmacist. These people can help you find information and resources that may be useful before, during and after treatment. Ask your doctor about the other members of your health care team – their names and how you can arrange to meet them. You also may want to ask your insurance company to assign you a case manager to help you understand which treatments and services your health insurance will cover.

Treatment choices depend on your diagnosis and the extent of the disease. The various drugs used to treat essential thrombocythemia work in different ways and may have different side effects. Ask for more information before starting treatment. Your options may include:

  • Aspirin, taken orally, in low doses, can help reduce the risk of clotting and is recommended for most ET patients.
  • Cytoreductive therapies (e.g. hydroxyurea, anagrelide), which may reduce the blood counts, are usually given by mouth (orally), and are recommended for patients considered high risk for blood counts (i.e. age > 60 or history of a prior blood clot).
  • Interferon alfa, which can lower blood counts, is given subcutaneously. Sustained release preparations (e.g. peginterferon alfa-2a), have superior tolerability.

Other treatments may be available through clinical trials. Clinical trials can offer the opportunity to benefit from the latest treatments or combinations of treatments while helping advance knowledge of the disease and its treatment options. Ask your health care team if a clinical trial might be right for you.