Polycythemia is a disease that can be determined by looking only at the 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 disease. Regardless of the chosen term, the disease poses a serious threat to human life. In this article, we will talk in more detail about the mechanism of its occurrence, primary symptoms, stages and proposed methods of treatment.
general information
True polycythemia refers to myeloproliferative blood cancer, in which the bone marrow produces red blood cells in excess. To a lesser extent, there is an increase in other enzyme elements, namely leukocytes and platelets.
Red blood cells (otherwise red blood cells) saturate all 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 the tissues and transporting it to the lungs for expiration.
Red blood cells are continuously produced in the bone marrow. It is a combination of spongy tissues, localized inside the bones and is responsible for the process of hematopoiesis.
White blood cells are white blood cells that help fight off various infections. Platelets are nuclear-free cell fragments that are activated when vascular integrity is impaired. They have the ability to stick together with each other and clog the hole, thereby stopping 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 is asymptomatic. According to studies, the average age of patients varies from 60 to approximately 79 years. Young people get sick much less often, but their illness is much harder. Representatives of the stronger sex, according to statistics, are several times more likely to be diagnosed with polycythemia.
Pathogenesis
Most of the health problems associated with this disease arise from a 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 arteries and veins. This situation often causes strokes and heart attacks. The thing is that thick blood flows several times slower through the vessels. The heart has to make more efforts to literally push it through.
Slowing blood flow does not allow internal organs to receive the necessary amount of oxygen. This entails the development of heart failure, headaches, angina pectoris, weakness and other health problems that are not recommended to be ignored.
Disease classification
- It lasts from 5 years or more.
- The spleen is of normal size.
- Blood tests show a moderate increase in the number of red blood cells.
- Complications are diagnosed extremely rarely.
- II A. Polycythemic stage.
- Duration from 5 to about 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 an increased content of all blood cells except lymphocytes.
- There is a tumor process in the spleen.
- In the clinical picture appears exhaustion, thrombosis, bleeding.
- In the bone marrow, a gradual formation of scars occurs.
- A sharp decrease in red blood cells, platelets, and white blood cells occurs in the blood.
- A marked increase in the size of the spleen and liver is observed.
- This stage, as a rule, develops 20 years after confirmation of the diagnosis.
- The disease can transform into acute or chronic leukemia.
Causes of the disease
Unfortunately, at present, experts cannot say exactly which factors lead to the development of a disease such as true polycythemia.
Most lean toward viral genetic theory. According to her, special viruses (there are about 15 in total) are introduced into the human body and, under the influence of some factors that adversely affect immune defense, penetrate into bone marrow cells and lymph nodes. Then these cells, instead of the necessary maturation, begin to rapidly divide and multiply, forming new fragments.
On the other hand, the cause of polycythemia can be hidden in a hereditary predisposition. Scientists have proven that close relatives of a sick person, as well as people with chromosome structure disorders, are more prone to this ailment.
Predisposing factors for the occurrence of the disease
- X-ray irradiation, ionizing radiation.
- Intestinal infections.
- Viruses.
- Tuberculosis.
- Surgical interventions.
- Frequent stresses.
- Long-term use of certain groups of drugs.
Clinical picture
Starting from the second stage of the development of the disease, literally all the systems of internal organs become involved in the pathological process. Below we list the subjective sensations of the patient.
- Weakness and a haunting feeling of fatigue.
- Increased sweating.
- Marked decrease in performance.
- Severe headaches.
- Memory impairment.
True polycythemia may also be accompanied by the following symptoms. In each case, their severity varies.
- Dilation of veins and a change in the shade of the skin. Patients report the presence of distinct dilated veins. With this disease, the skin has a reddish-cherry hue, it is especially noticeable on the open parts of the body (tongue, hands, face). Lips turn blue, eyes as if filled with blood. Such a change in appearance is explained by the overflow of superficial blood vessels with blood and a noticeable slowdown in its progress.
- Itchy skin. Such a symptom is observed in 40% of cases.
- Erythromelalgia (short-term burning pains on the tips of the fingers and toes, which are accompanied by redness of the skin). The occurrence of this symptom is associated with an increased content of platelets in the blood and the formation of microthrombi.
- An increase in the size 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.
- Blood clots. The reasons for their occurrence are explained by increased blood viscosity and changes in the vascular walls. Typically, this situation leads to circulatory disorders in the lower extremities, brain and coronary vessels.
- Pain in the legs.
- Heavy bleeding from the gums.
Diagnostics
First of all, the doctor collects a complete medical history. He can ask a number of clarifying questions: when exactly did the malaise / shortness of breath / painful discomfort, etc. It is equally important to determine the presence of chronic ailments, bad habits, possible contacts with toxic substances.
Then a physical examination is performed. The specialist determines the color of the skin. By palpation and tapping reveals an increase in the spleen or liver.
To confirm the disease, blood tests are mandatory. If the patient has this pathology, the test results may be as follows:
- An increase in the number of red blood cells.
- Increased hematocrit parameters (percentage of red blood cells).
- High rates of hemoglobin.
- Low levels of erythropoietin. This hormone is responsible for the stimulation of bone marrow during the production of new red blood cells.
Diagnosis also involves brain aspiration and biopsy. The first research option involves the sampling of the fluid part of the brain, and a biopsy of the solid component.
Polycythemia disease is confirmed by tests for gene mutation.
What should be the treatment?
It is not possible to completely overcome a disease such as true polycythemia. That is why therapy focuses solely on reducing clinical manifestations and reducing thrombotic complications.
Patients are first prescribed bloodletting. This procedure involves the removal of a small amount of blood (from 200 and up to about 400 ml) for therapeutic purposes. It is necessary to normalize the quantitative parameters of the blood and reduce its viscosity.
"Aspirin" is usually prescribed to patients to reduce the risk of developing various kinds of thrombotic complications.
Chemotherapy is used to maintain normal hematocrit when severe itching or increased thrombocytosis occurs.
Bone marrow transplantation in this disease is extremely rare, since this pathology in the case of adequate therapy is not deadly.
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:
- Blood clots. The causes of this pathological process may be hiding in an increase in blood viscosity, an increase in the number of red bodies, as well as platelets.
- Urolithiasis and gout.
- Bleeding even after minor surgery. As a rule, you have to face such a problem after tooth extraction.
Forecast
Wakez's disease is a rare disease. Symptoms that appear in the early stages of its development should be the reason for an 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 recommendations by the doctor 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. Be healthy!