What is it about ?
Polycythemia vera or polycythemia vera is a chronic disease of the blood, cancerous in appearance, which progresses gradually. It is characterized by an excess of blood cells in the bone marrow which produce red blood cells, white blood cells and platelets.
In polycythemia vera, hyperproduction involves red blood cells. Because red blood cells contain hemoglobin, there is an increase in hemoglobin levels in the results of a blood test.
Possible complications are bleeding and blood clots. Treatment helps prevent complications. The cause of this disease is not known.
Where and how often?
Each year, there are 2 new cases in 100,000 people. Most patients are between 50 and 70 years old.
How to recognize it?
Symptoms and possible signs are as follows:
- redness of the skin,
- redness of the conjunctivae (the mucous membrane that lines the inside of the eyelid and the white part of the eye),
- headaches and dizziness,
- itching,
- gastrointestinal symptoms, often bleeding,
- joint pain,
- nervous system symptoms: difficulty speaking, sudden loss of strength in the arms or legs, sensory disturbances, tingling,
- heavy bleeding or bruising,
- symptoms related to a blocked artery or vein, for example a swollen and painful leg, a chest pain.
In case of sudden signs, you should promptly contact the doctor.
How is the diagnosis made?
The doctor may suspect the diagnosis from your symptoms, physical examination, blood tests, and sometimes also medical imaging (for the detection of an enlarged spleen, for example).
The general practitioner will refer you to a doctor specializing in blood diseases (hematologist) to make the correct diagnosis. Most often, the hematologist will perform an examination of the bone marrow. It does this by extracting some bone marrow from your hip or other bone using a needle. This is necessary to make the correct diagnosis. The bone marrow is then looked at under a microscope to be able to study it and differentiate Polycythemia from other diseases.
What can you do ?
If you develop the symptoms mentioned above, contact the general practitioner. Ask him for explanations about the disease, and make sure you are familiar with the warning signs i.e. symptoms caused by a blood clot. Take your medicines well, such as aspirin (acetylsalicylic acid). If you develop any side effects from the treatment, talk to the doctor. Above all, do not stop the drug on your own.
What can the doctor do?
If you have this disease, it is very important that your doctor explains it clearly to you. This is because the possible complications of the disease can be serious and even life threatening. Getting enough information also increases your motivation to take the medicine correctly.
Treatment is aimed at avoiding serious complications due to blood clots or bleeding. Usually it works well. Treatment involves bleeding 400 to 500 ml, usually every two weeks, to remove excess blood cells. The hemoglobin level in the blood should be kept below a certain limit (14.5 g / dl). It is therefore sometimes necessary to collect more blood during bleeding.
If you need more than 4-8 bleeds per year, your doctor will consider prescribing medicine to slow cell growth as well. In younger patients, however, bleeding is avoided as much as possible.
To relieve the symptoms, the doctor will prescribe the following medications:
- antihistamines for itching, and antacids for stomach problems and itching. Sometimes the doctor will prescribe phototherapy to relieve the itching;
- interferon is sometimes used to slow cell growth, and is also effective against itching;
- allopurinol is given when the level of uric acid in the blood is too high to prevent gout and kidney damage.
Most often, people are also given aspirin (acetylsalicylic acid 100 mg) to prevent blood clots. However, aspirin increases the risk of bleeding.
Good follow-up is necessary because the disease progresses to an invasion of the bone marrow by fibrous tissue (fibrosis), and sometimes also to cancer of the white blood cells (acute leukemia). The general practitioner, in consultation with the specialist, will continue to follow you throughout your life.
Want to know more?
- Antihistamines – CBIP – Belgian Center for Pharmacotherapeutic Information
- Interferons – CBIP – Belgian Center for Pharmacotherapeutic Information
- Allopurinol – CBIP – Belgian Center for Pharmacotherapeutic Information
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