Polycythemia is a disease that can only be detected by looking at a person's face. And if you still conduct a diagnostic examination, then there will be no doubt at all. In the medical literature, you can find other names for this pathology: erythremia, Wakez's disease. Regardless of the term chosen, the disease carries a serious threat to human life. In this article, we will go into more detail about its mechanism of occurrence, primary symptoms, stages, and proposed treatments.
General information
Polycythemia vera is a myeloproliferative blood cancer in which the bone marrow produces red blood cells in excess amounts. To a lesser extent, there is an increase in other enzyme elements, namely leukocytes and platelets.
Red blood cells (otherwise erythrocytes) saturate all the cells of the human body with oxygen, delivering it from the lungs to the systems of internal organs. They are also responsible for removing carbon dioxide from tissues and transporting it to the lungs for exhalation.
RBCs are continuously produced in the bone marrow. He isa collection of spongy tissues, localized inside the bones and is responsible for the process of hematopoiesis.
Leukocytes are white blood cells that help fight various infections. Platelets are non-nuclear cell fragments that are activated when the integrity of blood vessels is violated. They have the ability to stick together and clog the hole, thereby stopping the bleeding.
Polycythemia vera is characterized by excessive production of red blood cells.
Disease prevalence
This pathology is usually diagnosed in adult patients, but can occur in adolescents and children. For a long time, the disease may not make itself felt, that is, it may be asymptomatic. According to studies, the average age of patients varies from 60 to approximately 79 years. Young people get sick much less often, but the disease is much more severe for them. The representatives of the stronger sex, according to statistics, are several times more likely to be diagnosed with polycythemia.
Pathogenesis
Most of the he alth problems associated with this disease are due to the continuous increase in the number of red blood cells. As a result, the blood becomes excessively thick.
On the other hand, its increased viscosity provokes the formation of clots (blood clots). They can interfere with normal blood flow through the arteries and veins. This situation often causes strokes and heart attacks. The whole point is thatthick blood flows several times slower through the vessels. The heart has to work harder to literally push it through.
Slowing blood flow does not allow the internal organs to receive the required amount of oxygen. This entails the development of heart failure, headaches, angina pectoris, weakness and other he alth problems that are not recommended to be ignored.
Disease classification
I. Initial stage
- Lasts 5 years or more.
- Spleen of normal size.
- Blood tests show a moderate increase in red blood cells.
- Complications are extremely rare.
II A. Polycythemic stage
- Duration 5 to approximately 15 years.
- There is an increase in some organs (spleen, liver), bleeding and thrombosis.
- There are no areas of the tumor process in the spleen itself.
- Bleeding can cause iron deficiency in the body.
- In the blood test, there is a persistent increase in red blood cells, white blood cells and platelets.
II B. Polycythemic stage with myeloid metaplasia of the spleen
- Tests show elevated levels of all blood cells except lymphocytes.
- There is a tumor process in the spleen.
- The clinical picture shows emaciation, thrombosis, bleeding.
- The bone marrow is gradually scarred.
III. Anemic stage
- In the blood there is a sharpdecrease in red blood cells, platelets and white blood cells.
- There is a pronounced increase in the size of the spleen and liver.
- This stage usually develops 20 years after the diagnosis is confirmed.
- The disease can transform into acute or chronic leukemia.
Causes of disease
Unfortunately, at present, experts cannot say which factors lead to the development of such a disease as polycythemia vera.
Most lean towards the virus-genetic theory. According to her, special viruses (there are about 15 of them in total) are introduced into the human body and, under the influence of certain factors that negatively affect immune defense, penetrate into the cells of the bone marrow and lymph nodes. Then, instead of maturing, these cells begin to rapidly divide and multiply, forming more and more new fragments.
On the other hand, the cause of polycythemia may be hidden in hereditary predisposition. Scientists have proven that close relatives of a sick person, as well as people with violations of the structure of chromosomes, are more susceptible to this disease.
Predisposing factors to disease
- X-ray exposure, ionizing radiation.
- Intestinal infections.
- Viruses.
- Tuberculosis.
- Surgical interventions.
- Frequent stress.
- Long-term use of certain drug groups.
Clinical picture
Starting from the second stepdevelopment of the disease, literally all systems of internal organs are involved in the pathological process. Below we list the subjective feelings of the patient.
- Weakness and haunting tiredness.
- Excessive sweating.
- Noticeable performance degradation.
- Severe headaches.
- Memory deterioration.
Polycythemia vera may also be accompanied by the following symptoms. In each case, their severity varies.
- Expansion of veins and change in skin tone. Patients note the presence of clearly defined dilated veins. With this disease, the skin is distinguished by a reddish-cherry tint, it is especially noticeable on open parts of the body (tongue, hands, face). The lips become blue, the eyes seem to be filled with blood. Such a change in appearance is explained by the overflow of superficial vessels with blood and a noticeable slowdown in its progress.
- Skin itching. This symptom occurs in 40% of cases.
- Erythromelalgia (short-term burning pain on the tips of the fingers and toes, accompanied by reddening of the skin). The occurrence of this symptom is associated with an increased content of platelets in the blood and the formation of microthrombi.
- Enlargement of the spleen.
- The appearance of ulcers in the stomach. Due to thrombosis of small vessels, the mucous membrane of the organ loses its resistance to Helicobacter pylori.
- Clot formation. The reasons for their occurrence are explained by increased blood viscosity and changes in the vascular walls. Typically, this situationleads to circulatory disorders in the lower extremities, cerebral and coronary vessels.
- Pain in the legs.
- Gum bleeding profusely.
Diagnosis
First of all, the doctor collects a complete history. He can ask a number of clarifying questions: when exactly the malaise / shortness of breath / painful discomfort appeared, etc. It is equally important to determine the presence of chronic ailments, bad habits, possible contact with toxic substances.
Then a physical examination is done. The specialist determines the color of the skin. By palpation and tapping, it reveals an enlarged spleen or liver.
Blood tests are mandatory to confirm the disease. If the patient has this pathology, the test results may be as follows:
- Increase in the number of red blood cells.
- Elevated hematocrit (percentage of red blood cells).
- High hemoglobin.
- Low erythropoietin levels. This hormone is responsible for stimulating the bone marrow to produce new red blood cells.
Diagnosis also includes brain aspiration and biopsy. The first version of the study involves taking the liquid part of the brain, and a biopsy - the solid component.
Polycythemia disease is confirmed by gene mutation tests.
What should be the treatment?
Completely overcome the disease like polycythemiatrue, not possible. That is why therapy focuses solely on reducing clinical manifestations and reducing thrombotic complications.
Patients are given bloodletting first. This procedure involves the removal of a small amount of blood (from 200 to approximately 400 ml) for therapeutic purposes. It is necessary to normalize the quantitative parameters of blood and reduce its viscosity.
Patients are usually prescribed "Aspirin" to reduce the risk of developing various kinds of thrombotic complications.
Chemotherapy is used to maintain a normal hematocrit when there is severe itching or increased thrombocytosis.
Bone marrow transplantation in this disease is extremely rare, since this pathology is not deadly in case of adequate therapy.
It should be noted that the specific treatment regimen in each case is selected individually. The above therapy is for informational purposes only. It is not recommended to try to cope with this disease on your own.
Possible Complications
This disease is quite serious, so do not neglect its treatment. Otherwise, the likelihood of unpleasant complications increases. These include the following:
- Clot formation. The causes of this pathological process may be hidden in an increase in blood viscosity, an increase in the number of red cells, as well as platelets.
- Urolithiasis and gout.
- Bleeding even after minor surgery. As a rule, such a problem is encountered after tooth extraction.
Forecast
Wakez disease is a rare disease. Symptoms that appear in the early stages of its development should be the reason for immediate examination and subsequent therapy. In the absence of adequate treatment, if the disease was not diagnosed in a timely manner, death occurs. The main cause of death is most often vascular complications or the transformation of the disease into chronic leukemia. However, competent therapy and strict adherence to all the doctor's recommendations can significantly extend the patient's life (by 15-20 years).
We hope that all the information presented in the article will be really useful for you. Stay he althy!
