What Is Essential Thrombocythemia?
- Essential thrombocythemia (ET) is a rare type of blood cancer in which your bone marrow makes a large number of platelets. Too many platelets can make your blood thick and can cause problems in the clotting process.
- In the early stage of the disease, you may not have any symptoms; a routine blood test at your doctor’s office may show an abnormally higher level of platelets.
- Your doctor will determine your ET treatment based on age, symptoms, presence of mutations, or blood clots. If you are older than 60 years and have a history of a blood clot, medicines such as hydroxyurea are often recommended by the doctor to keep your bone marrow from making too many platelets.
In our body platelets serve a very important function, which is to seal off a leaking blood vessel and control bleeding after an injury or trauma. Too many platelets can make your blood thick and cause problems in the clotting process. There are other conditions that can also increase the platelet count in your body, such as inflammatory conditions or iron deficiency anemia. Your doctor will check your labwork to exclude these conditions before reaching the confirmed diagnosis of ET.
What is Essential Thrombocythemia (ET)?Read More
What are the symptoms of Essential Thrombocythemia (ET)?In the early stage of the disease, you may not have any symptoms; a routine blood test at your doctor’s office may show an abnormally high level of platelets.
In general, the common symptoms include:
- Frequent headaches
- Profuse sweating, particularly at night
- Painful, swollen joints of hands or feet
- Symptoms of a blood clot such as pain, swelling, redness, and warmth in the legs or arms
- Unexpected bleeding
What are the Complications of Essential Thrombocythemia (ET)?
One of the major complications you may experience with ET is the development of blood clots. Blood clots can occur in the deep veins of the legs and arms (known as DVT or deep vein thrombosis). These blood clots can break off from the vein and travel to another organ system. Blood clots in the brain (causing a stroke), eyes (causing blindness), heart (causing a heart attack), and lung (pulmonary embolism) are the most dangerous complications of ET. There is no definitive platelet number in your blood that your doctor can predict the risk of thrombosis.
In pregnancy, ET can cause problems in the placenta or fetus. This may affect fetal growth and can lead to a miscarriage.
If your platelet count reaches more than a million, you may also experience abnormal bleeding such as nosebleeds, bleeding from the mouth or gum, blood in the stool or bruises on the skin. It may also take longer for the bleeding to stop after a minor cut or injury.
How is Essential Thrombocythemia (ET) Diagnosed?
ET is generally discovered incidentally at the doctor’s office after a routine blood test or if you develop a blood clot. Your doctor will come to a diagnosis on the basis of the following tests:
- Complete blood count (CBC): This routine blood tests checks the levels of hemoglobin, different types of white blood cells, and platelets. In ET, your platelet count is higher than normal. Hemoglobin and white cell count will help to exclude other causes of the elevated platelet count.
- Blood smear: Blood samples are examined under a microscope to rule out other types of blood cancer such as acute leukemia.
- Bone marrow examination: Bone marrow examination (also known as bone marrow biopsy) may be needed in some patients to confirm the diagnosis. Your doctor takes a small sample of your bone marrow, usually from the hip bone. Then the pathologist looks at the cells under a microscope to determine cancer, presence of mutation, and/or chromosomal abnormality on the cancer cells.
How is Essential Thrombocythemia (ET) Treated?
Your doctor will determine your ET treatment based on age, symptoms, presence of mutations, or blood clots. Dr. Adrienne Phillips, a hematologist/oncologist at Weill Cornell Medicine explained the goal of the treatment: “Patients with essential thrombocythemia need therapy to lower the counts.”
- If you are younger than 60 years old and have no history of blood clots, your doctor may prescribe low-dose aspirin to keep your platelets from sticking together and to prevent blood clots. Also, your doctor may advise you on lifestyle modifications such as stopping smoking, maintaining a healthy weight range, and controlling blood pressure.
- If you are older than 60 years and have a history of blood clots, medicines such as hydroxyurea are often recommended to keep your bone marrow from making too many platelets. Hydroxyurea is an oral chemotherapy medication that decreases the production of platelets by your bone marrow. If hydroxyurea is not helpful in managing symptoms or is causing you too many side effects such as nausea, vomiting, diarrhea, leg ulcers, flu-like symptoms, bone pain, and loss of appetite, your doctor may change the medicine to Anagrelide (Agrylin) or Interferon alfa-2b (Intron A). Anagrelide is an oral medication that decreases platelet production in your bone marrow, thereby reducing symptoms and the risk of a blood clot. Interferon is given by injection and works by decreasing the specialized platelet-forming cells in your bone marrow.
- If you are pregnant or want to become pregnant, you should discuss the treatment goals with your obstetrician and hematologist. Doctors generally prefer interferon over hydroxyurea or anagrelide during pregnancy due to the potential harmful effect on the fetus. You may also need to take aspirin or blood thinner during your pregnancy to prevent a miscarriage.
Unfortunately, there is no cure for ET, but most patients with ET have a normal life span. You should follow up closely with your doctor for regular blood work and physical exams to prevent long-term complications.
Questions To Ask Your Doctor
- What is the best way to manage my ET symptoms?
- Are there side effects to consider while treating ET?
- How do we monitor the progress of my ET?
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