Primary polycythemia; Polycythemia rubra vera; P. Vera; Myeloproliferative disorder; Erythremia; Splenomegalic polycythemia; Vaquez’s disease; Osler’s disease; Polycythemia with chronic cyanosis; Erythrocytosis megalosplenica; Cryptogenic polycythemia
Polycythemia vera — is a chronic blood disease, in which the body produces too many red blood cells (red blood cells). Sometimes the number of white blood cells and platelets also increases. This condition belongs to a group of so-called myeloproliferative diseases., that is, diseases, associated with bone marrow dysfunction.
Bone marrow is the tissue inside bones, which is responsible for the production of blood cells. Normally, it produces exactly the same number of cells, how much does the body need. However, with polycythemia vera, this process gets out of control.. As a result, the blood becomes thicker, than usual, which slows its movement through the vessels.
Increased blood viscosity is a key problem in this disease. It can lead to blood clots (blood clots), which are capable of blocking blood vessels. It, in turn, increases the risk of serious complications, such as stroke, heart attack or deep vein thrombosis.
The disease develops slowly. For many people, it can go almost unnoticed for a long time.. Sometimes the diagnosis is made by chance - for example, with a routine blood test, when an elevated level of hemoglobin or hematocrit is detected.
True polycythemia is more often found in adults, especially those over 50–60 years old, although in rare cases it can also develop in younger patients. Men are slightly more likely to get it, than women.
It is important to understand, that this is a chronic disease, that is, it is difficult to completely cure it, but with proper treatment, symptoms can be controlled and the risk of complications can be significantly reduced. Many people with this diagnosis live long and active lives., if they follow the doctor's recommendations.
It is also worth noting, that true polycythemia can over time progress to more serious conditions, eg, myelofibrosis or acute leukemia. However, this does not occur in all patients and usually with a long course of the disease..
Thus, Polycythemia vera is not just “thick blood”, and a complex disease, requiring careful monitoring and regular medical supervision.
The main cause of the development of true polycythemia is a genetic mutation. In most cases, we are talking about a mutation in the JAK2 gene. This gene is responsible for transmitting signals inside bone marrow cells, which regulate the growth and division of blood cells.
When does mutation occur?, cells begin to receive a “signal to grow” even then, when it's not necessary. As a result, the bone marrow begins to produce too many red blood cells, and sometimes other blood cells.
It is important to note, that this mutation is usually not inherited. It occurs during a person's life, that is, it is acquired. Therefore, most patients do not have relatives with the same disease.
Why this mutation occurs exactly is not fully known. However, there are factors, which can increase the risk of developing the disease:
Sometimes polycythemia is confused with conditions, in which the number of red blood cells increases for other reasons - for example, due to dehydration or chronic lack of oxygen (in case of lung diseases or living at high altitude). However, in these cases it is not about true polycythemia, but about secondary.
True polycythemia differs in that, that the problem is in the bone marrow. This is an independent disease, and not the body's reaction to external conditions.
Understanding the cause of the disease is important for choosing the right treatment. For Example, Modern drugs can specifically target the disrupted signaling pathways within cells, slowing down their excessive proliferation.
Although it is impossible to prevent the occurrence of mutations, early diagnosis allows you to start treatment on time and avoid serious complications.
Symptoms of polycythemia vera can vary and often develop gradually. In the early stages, a person may not notice any changes at all.
One of the most characteristic signs is a feeling of fatigue. It may be permanent and not go away even after rest.. Patients also often complain of headaches and dizziness..
Thick blood can cause circulatory problems. This is manifested by the following symptoms:
A very characteristic symptom is itching of the skin, especially after a hot shower or bath. This is due to changes in blood composition and skin reaction.
May also be observed:
The most dangerous manifestations are associated with the formation of blood clots. They can lead to serious complications:
Sometimes, conversely, there is a tendency to bleed - for example, nasal or gum.
It is important to remember, that symptoms may vary from person to person. Some people have almost asymptomatic disease, and for some it manifests itself clearly in the early stages.
If unusual symptoms occur, especially related to blood circulation, It is important not to delay seeing a doctor.
You should consult a doctor if any suspicious symptoms appear, especially if they persist for a long time or intensify.
Reasons for consultation may be:
It is especially important to seek immediate medical attention if there are signs of thrombosis.:
These symptoms may indicate a stroke or heart attack and require immediate attention.
Even if the symptoms seem minor, it's better to check your health condition. Sometimes a disease is discovered accidentally during a blood test, and this provides an opportunity to start treatment at an early stage.
Regular preventive examinations are especially important for older people.
At the appointment, the doctor will try to collect as much information as possible about the patient’s condition..
He can ask the following questions:
The doctor may also ask about the patient's lifestyle:
These questions help to assess risk factors and choose the optimal strategy for diagnosis and treatment.
Diagnosis begins with a general blood test. The doctor pays attention to the hemoglobin level, hematocrit and blood cell count.
If the indicators are elevated, additional studies are prescribed:
A biopsy allows assessing the condition of the bone marrow and confirming the diagnosis.
Studies can also be conducted to evaluate the risk of thrombosis.
Diagnosis requires an integrated approach and is carried out under the supervision of a hematologist..
Treatment is aimed at reducing blood density and preventing complications.
Basic methods:
The choice of treatment depends on the patient's age, symptoms and risk of complications.
Therapy is usually long-term and requires regular monitoring.
Home measures help improve the condition and reduce the risk of complications:
It is important to follow the doctor's recommendations and not self-medicate.
There is no specific prevention, since the disease is associated with a mutation.
However, you can reduce the risk of complications:
Early diagnosis and proper treatment are the key to maintaining health.
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