Polycythemia is true. Causes, symptoms, diagnosis, treatment

Polycythemia is a disease that can be identified 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, Vaquez disease. Regardless of the term chosen, the disease carries a serious threat to human life. In this article we will describe in more detail the mechanism of its occurrence, primary symptoms, stages and proposed methods of treatment.

general information

True polycythemia is understood as myeloproliferative blood cancer, in which the bone marrow produces red blood cells in excess quantities. To a lesser extent, other enzyme elements, namely leukocytes and platelets, increase.

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.

Erythrocytes are continuously produced in the bone marrow. It is a collection of spongiform tissues, localized inside the bones and responsible for the process of hematopoiesis.

Leukocytes are white blood cells that help fight various infections. Platelets are denuclearized cell fragments that activate in vascular integrity disorders. They have the ability to stick together and clog the hole, thus stopping bleeding.

Polycythemia true is characterized by excessive production of red blood cells.

Prevalence of the disease

This pathology is usually diagnosed in adult patients, but can occur in adolescents and children. For a long time, the ailment can not make itself felt, that is, it is asymptomatic. According to the research, the average age of patients varies from 60 to about 79 years. Young people get sick less often, but they have a much harder illness. In representatives of the stronger sex, according to statistical data, polycythemia is diagnosed several times more often.

Pathogenesis

Most of the health problems associated with this disease occur 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 (thrombi). They can interfere with normal blood flow through the arteries and veins. This situation often causes strokes and heart attacks. The thing is that thick blood flows several times more slowly through the blood vessels. The heart has to make more efforts to literally push it.

Slowdown 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.

Classification of the disease

• I. The initial stage.

1. Lasts from 5 years or more.
2. The spleen is of normal size.
3. Blood tests show a moderate increase in the number of red blood cells.
4. Complications are rarely diagnosed.

• II A. Polycythemic stage.

1. Duration from 5 to 15 years.
2. There is an increase in some organs (spleen, liver), bleeding and thrombosis.
3. There are no parts of the tumor process in the spleen itself.
4. Bleeding can provoke a deficiency of iron in the body.
5. In the analysis of blood there is a persistent increase in erythrocytes, leukocytes and platelets.

• II B. Polycythemic stage with myeloid metaplasia of the spleen.

1. Analyzes show an increased content of all blood cells, except for lymphocytes.
2. There is a tumor process in the spleen.
3. In the clinical picture there is depletion, thrombosis, bleeding.
4. In the bone marrow there is a gradual formation of scars.

• III. Anemic stage.

1. In the blood there is a sharp decrease in erythrocytes, platelets and leukocytes.
2. There is a pronounced increase in the size of the spleen and liver.
3. This stage, as a rule, develops 20 years after the confirmation of the diagnosis.
4. The disease can be transformed into acute or chronic leukemia.

Causes of the disease

Unfortunately, at present specialists can not say which factors lead to the development of such a disease as polycythemia is true.

Most tend to the viral-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 some factors that adversely affect the immune defense penetrate into the cells of the bone marrow and lymph nodes. Then, instead of the proper maturation, these cells begin to rapidly divide and multiply, forming new fragments.

On the other hand, the cause of polycythaemia may be hiding in a hereditary predisposition. Scientists have proved that this ailment is more susceptible to close relatives of a sick person, as well as people with impaired chromosome structure.

Predisposing to the appearance of the disease factors

• X-ray irradiation, ionizing radiation.
• Intestinal infections.
• Viruses.
• Tuberculosis.
• Surgical interventions.
• Frequent stress.
• Long-term use of certain groups of drugs.

Clinical picture

Beginning with the second stage of the development 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 a haunting feeling of fatigue.
• Increased sweating.
• A marked decrease in efficiency.
• Severe headaches.
• Memory impairment.

Polycythemia true can also be accompanied by the following symptoms. In each case, their severity varies.

• Vein enlargement and skin tint change. Patients report the presence of distinctive enlarged veins. With this disease, the skin is characterized by a reddish-cherry tint, especially in the open parts of the body (tongue, hands, face). Lips turn blue, eyes like blood. This change in appearance is due to overfilling of the surface vessels with blood and a marked slowing of its progress.
• Itching itch. This symptom is observed in 40% of cases.
• Erythromelalgia (short-term burning pains on the tips of the fingers and toes, which are accompanied by reddening of the skin). The emergence of this symptom is associated with an increased content of platelets in the blood and the formation of microthrombi.
• Increase in the size of the spleen.
• The appearance of ulcers in the stomach. Because of the 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 the increased viscosity of the blood and changes in the vascular walls. As a rule, such a situation leads to a violation of blood circulation in the lower limbs, brain and coronary vessels.
• Pain in the legs.
• Abundant bleeding from the gums.

Diagnostics

First of all the doctor collects the full anamnesis. He can ask a number of clarifying questions: when exactly there was malaise / dyspnea / 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 carried out. The specialist determines the color of the skin. Through 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 can be as follows:

• Increase in the number of red blood cells.
• Elevated parameters of hematocrit (percentage of red blood cells).
• High indicators of hemoglobin.
• Low level of erythropoietin. This hormone is responsible for stimulating the bone marrow in the process of producing new red blood cells.

Diagnosis also involves aspiration of the brain and a biopsy. The first variant of the study involves sampling the liquid part of the brain, and a biopsy - a solid component.

The disease polycythemia is confirmed by tests for the gene mutation.

What should be the treatment?

Completely overcome such a disease as polycythemia is true, it is not possible. That is why therapy focuses exclusively on reducing clinical manifestations and reducing thrombotic complications.

In the first place, patients are given bloodletting. This procedure involves the removal of a small amount of blood (from 200 and approximately 400 ml) for a therapeutic purpose. It is necessary to normalize the quantitative parameters of blood and reduce its viscosity.

Patients usually prescribe "Aspirin" to reduce the risk of developing various kinds of thrombotic complications.

Chemotherapy is used to maintain normal hematocrit when there is severe itching or increased thrombocytosis.

Bone marrow transplantation in this disease is extremely rare, since this pathology in case of adequate therapy is not deadly.

It is necessary to notice, the concrete scheme or plan of treatment in each case is selected individually. The above-described therapy is only for informational purposes. It is not recommended to try to cope with this disease on your own.

Possible complications

This disease is serious enough, so do not neglect his treatment. Otherwise, the probability of unpleasant complications increases. These include the following:

• Blood clots. The causes of this pathological process can be hidden in the increase of blood viscosity, increase in the number of red bodies, and also platelets.
• Urolithiasis and gout.
• Bleeding even after minor surgical interventions. As a rule, you have to deal with such a problem after tooth extraction.

Forecast

Vaquez disease is a rare disease. Symptoms that appear in the early stages of its development should be an occasion for immediate examination and subsequent therapy. In the absence of adequate treatment, if the disease has not been diagnosed in a timely manner, death occurs. The main cause of death is most often vascular complications or transformation of the disease into chronic leukemia. However, competent therapy and strict adherence to all the recommendations of the doctor can significantly prolong the patient's life (by 15-20 years).

We hope that all the information presented in this article will be really useful for you. Be healthy!