- What are the signs and symptoms
- How the diagnosis is made
- Complications of polycythemia vera
- 1. Formation of blood clots
- 2. Splenomegaly
- 3. Occurrence of other diseases
- How to prevent complications
- Possible causes
- How the treatment is done
Polycythemia Vera is a myeloproliferative disease of hematopoietic cells, which is characterized by an uncontrolled proliferation of red blood cells, white blood cells and platelets.
The increase in these cells, especially red blood cells, makes the blood thicker, which can lead to other complications such as enlarged spleen and increased blood clots, thus increasing the risk of thrombosis, heart attack or stroke or even causing other diseases like acute myeloid leukemia or myelofibrosis.
Treatment consists of performing a procedure called phlebotomy and administering drugs that help regulate the number of cells in the blood.
What are the signs and symptoms
The high number of red blood cells causes an increase in hemoglobin and blood viscosity, which can cause neurological symptoms such as vertigo, headache, increased blood pressure, visual changes and transient ischemic accidents.
In addition, people with this disease often experience generalized itching, especially after a hot bath, weakness, weight loss, tiredness, blurred vision, excessive sweating, joint swelling, shortness of breath and numbness, tingling, burning or weakness in the members.
How the diagnosis is made
In order to make the diagnosis of the disease, blood tests must be carried out, which in people with Polycythemia Vera, show an increase in the number of red blood cells, and in some cases, an increase in white blood cells and platelets, high levels of hemoglobin and low levels of erythropoietin.
In addition, a bone marrow aspiration or biopsy can also be performed in order to obtain a sample to be analyzed later.
Complications of polycythemia vera
There are some cases of people with Polycythemia Vera who do not show signs and symptoms, however, some cases can give rise to more serious problems:
1. Formation of blood clots
The increase in the thickness of the blood and the consequent decrease in the flow and change in the number of platelets, can cause the formation of blood clots, which can lead to a heart attack, stroke, pulmonary embolism or thrombosis. Learn more about cardiovascular disease.
2. Splenomegaly
The spleen helps the body to fight infections and helps to eliminate damaged blood cells. The increase in the number of red blood cells or even other blood cells, makes the spleen have to work harder than normal, leading to an increase in size. See more about splenomegaly.
3. Occurrence of other diseases
Although rare, Polycythemia Vera can give rise to other more serious diseases, such as myelofibrosis, myelodysplastic syndrome or acute leukemia. In some cases, the bone marrow may also develop progressive fibrosis and hypocellularity.
How to prevent complications
To prevent complications, besides being recommended to follow the treatment correctly, it is also important to adopt a healthier lifestyle, practicing physical exercise regularly, which improves blood circulation and reduces the risk of blood clots. Smoking should also be avoided, as it increases the risk of heart attack and stroke.
In addition, the skin should be treated well, in order to reduce itching, taking a bath with warm water, using a mild shower gel and a hypoallergenic cream and avoiding extreme temperatures, which can worsen blood circulation. For this, one should avoid sun exposure in hot periods of the day and protect the body from exposure to very cold weather.
Possible causes
Polycythemia Vera occurs when a JAK2 gene is mutated, which causes problems in the production of blood cells. This is a rare disease, which occurs in about 2 in every 100, 000 people, usually over the age of 60.
Usually the healthy organism regulates the amount of production of each of the three types of blood cells: red, white blood cells and platelets, but in Polycythemia Vera, there is an exaggerated production of one or more types of blood cells.
How the treatment is done
Polycythemia vera is a chronic disease that cannot be cured and treatment consists of reducing the excess blood cells, and in some cases can reduce the risk of complications:
Therapeutic phlebotomy: This technique consists of draining blood from the veins, which is usually the first treatment option for people with this disease. This procedure reduces the number of red blood cells, also reducing the volume of blood.
Aspirin: The doctor may prescribe aspirin in a low dose, between 100 and 150 mg, to reduce the risk of blood clots.
Medicines to decrease blood cells: If phlebotomy is not sufficient for the treatment to be effective, it may be necessary to take medications such as:
- Hydroxyurea, which can reduce the production of blood cells in the bone marrow; Interferon alfa, which stimulates the immune system to fight against the excessive production of blood cells, for people who do not respond well to hydroxyurea; Ruxolitinib, which helps the immune system to destroy tumor cells and can improve symptoms; Medicines to reduce itching, such as antihistamines.
If the itch becomes very severe, it may be necessary to have ultraviolet light therapy or use medications such as paroxetine or fluoxetine.
