Polycythemia is a chronic disease in which the amount of red blood cells (erythrocytes) in the blood increases. Also, with this pathology, in 70% of patients, the number of platelets and leukocytes changes upwards.
The disease does not have a high prevalence - no more than five cases are recorded annually per one million population. Most often, polycythemia develops in middle-aged and elderly people. According to statistics, males suffer from this pathology five times more often than women. Today we will take a closer look at a condition such as polycythemia, the symptoms and treatment of the pathology will be described below.
Causes of disease development
Polycythemia is not a malignant disease. To date, the exact causes of the disease are unknown. It is believed that the development of pathology is caused by a mutation of a special enzyme in the bone marrow. Gene changes lead to excessive division and growth of all blood cells, and especially red blood cells.
Disease classification
There are two groups of illness:
- Truepolycythemia, or Wakez's disease, which in turn is divided into primary (that is, acts as an independent disease) and secondary (secondary polycythemia develops due to chronic lung diseases, tumors, hydronephrosis, climbing to a height).
- Relative polycythemia (stress or false) - in this condition, the level of red blood cells remains within the normal range.
Polycythemia: symptoms of the disease
Very often the disease is asymptomatic. Sometimes, as a result of an examination for completely different reasons, polycythemia vera can be accidentally detected. See below for symptoms to look out for.
Dilation of the saphenous veins
With polycythemia on the skin, most often in the neck, dilated saphenous veins appear. With such a pathology, the skin becomes a reddish-cherry hue, this is especially noticeable in open areas of the body - the neck, hands, face. The mucous membrane of the lips and tongue has a bluish-red color, the whites of the eyes seem to be bloodshot.
Itchy skin
Almost half of patients with polycythemia develop severe itching, especially after taking a warm bath. Such a phenomenon appears inas a specific sign of polycythemia vera. Itching occurs due to the release of active substances into the blood, in particular histamine, which is able to expand skin capillaries, which leads to increased blood circulation in them and the appearance of specific sensations.
Erythromelalgia
This phenomenon is characterized by short-term severe pain in the area of the fingertips. It provokes their increase in the level of platelets in the small vessels of the hand, resulting in the formation of numerous microthrombi that clog arterioles and block the flow of blood to the tissues of the fingers. External signs of this condition are redness and the appearance of cyanotic spots on the skin. Aspirin is recommended to prevent thrombosis.
Splenomegaly (enlarged spleen)
In addition to the spleen, the liver can also change, or rather, its size. These organs are directly involved in the formation and destruction of blood cells. An increase in the concentration of the latter leads to an increase in the size of the liver and spleen.
Ulcers of the duodenum and stomach
Such a serious surgical pathology develops as a result of thrombosis of small vessels of the mucous membrane of the digestive tract. The result of an acute circulatory disorder is the necrosis (necrosis) of a section of the organ wall and the formation of an ulcer in its place. In addition, the resistance of the stomach to Helicobacter (a microorganism that causes gastritis and ulcers) is reduced.
Thrombi in large vessels
The veins of the lower extremities are more prone to this pathology. Thrombi, breaking away from the wall of the vessel, can, bypassing the heart, penetrate into the pulmonary circulation (lungs) and provoke PE (pulmonary embolism) - a condition incompatible with life.
Bleeding gums
Despite the fact that the number of platelets in the peripheral blood changes and its clotting increases, gingival bleeding can occur with polycythemia.
Gout
When the level of uric acid rises, its s alts are deposited in various joints and provoke a sharp pain syndrome.
- Pain in limbs. This symptom causes damage to the arteries of the legs, their narrowing and, as a result, impaired blood circulation. This pathology is called "obliterating endarteritis"
- Pain in flat bones. Increased activity of the bone marrow (where blood cells develop) provokes the sensitivity of flat bones to mechanical stress.
Deterioration of the general condition of the body
With a disease such as polycythemia, the symptoms may be similar to signs of other pathologies (for example, anemia): headaches, constant fatigue, tinnitus, dizziness, goosebumps before the eyes, shortness of breath, flushing head. An increase in the viscosity properties of the blood activates the compensatory reaction of the vessels, as a result, an increase in blood pressure occurs. With this pathology, complications are often observed in the form of heart failure and microcardiosclerosis (replacement of muscleconnective tissue of the heart, filling the defect, but not performing the necessary functions).
Diagnosis
Polycythemia is detected by the results of a general blood test, which shows:
- increased red cell count from 6.5 to 7.5•10^12/L;
- increased hemoglobin level - up to 240 g/l;
- total erythrocyte volume (RBC) exceeds 52%.
Since the number of erythrocytes cannot be calculated based on the measurements of the above values, radionuclide diagnostics is used for measurement. If the mass of red blood cells exceeds 36 ml / kg in men and 32 ml / kg in women, then this is a reliable indication of the presence of Wakez disease.
With polycythemia, the morphology of erythrocytes is preserved, that is, they do not change their normal shape and size. However, with the development of anemia as a result of increased bleeding or frequent bloodletting, microcytosis (decrease in red blood cells) is observed.
Polycythemia treatment
Bloodletting has a good therapeutic effect. It is recommended to remove 200-300 ml of blood weekly until the TBE level drops to the desired value. If there are contraindications for bloodletting, it is possible to restore the percentage of erythrocytes by diluting blood by adding a liquid part to it (high molecular weight solutions are administered intravenously).
It should be borne in mind that quite often bloodletting leads tothe development of iron deficiency anemia, in which there are corresponding symptoms and an increase in platelets.
With such an ailment as polycythemia vera, treatment involves following a certain diet. It is recommended to limit the consumption of meat and fish products, as they contain a high amount of protein, which actively stimulates the activity of the blood-forming organs. You should also avoid fatty foods. Cholesterol contributes to the development of atherosclerosis, resulting in blood clots, which are already formed in large numbers in people suffering from polycythemia.
With such a disease, it is recommended to give preference to dairy and vegetable products, as well as limit physical activity.
Also, if the diagnosis is polycythemia, treatment may include chemotherapy. Apply it with increased thrombocytosis and severe itching. As a rule, this is a “cytoreductive agent” (drug “Hydroxycarbamide”).
Until recently, injections of radioactive isotopes (usually phosphorus-32) were used to suppress the bone marrow. Today, such treatment is increasingly being abandoned due to the high rate of leukemic transformation.
Therapy also includes injections of interferon, in the treatment of secondary thrombocytosis, the drug "Anagrelide" is used.
With this pathology, bone marrow transplantation is very rarely performed, since polycythemia is a disease that is not fatal, provided, of course, adequate treatment and constantcontrol.
Polycythemia in newborns
Polycythemia is a pathology, the symptoms of which can be found in newborns. This disease is a response of the body of the crumbs to the transferred hypoxia, which could be provoked by placental insufficiency. The baby's body begins to synthesize a large number of red blood cells to correct hypoxia.
In addition to the respiratory condition, newborns can also develop polycythemia vera. Twins are particularly at risk.
Polycythemia in a newborn develops in the first weeks of life, its first manifestations are an increase in hematocrit (up to 60%) and a significant increase in hemoglobin levels.
Neonatal polycythemia has several stages of flow: the initial, the stage of proliferation and depletion. Let's briefly describe them.
The initial stage of the disease has practically no clinical manifestations. It is possible to detect polycythemia in a child at this stage only by examining peripheral blood parameters: hematocrit, hemoglobin and red blood cells.
At the stage of proliferation, an increase in the liver and spleen develops. Plethoric phenomena are observed: the skin acquires a characteristic "plethoric-red" shade, the child shows anxiety when touching the skin. Plethoric syndrome is complemented by thrombosis. In the analyzes, there is a change in the number of erythrocytes, platelets and leukocyte shifts. All blood cell counts may also increase, suchthe phenomenon is called "panmyelosis".
The wasting stage is characterized by significant weight loss, asthenia and emaciation.
For a newborn, such clinical changes are extremely severe and can provoke irreversible changes and subsequent death. Polycythemia can cause a malfunction in the production of certain types of white blood cells, which are responsible for the body's immune system. As a result, the infant develops severe bacterial infections, eventually leading to death.
After reading this article, you learned more about such a pathology as polycythemia. Symptoms and treatment have been considered by us in as much detail as possible. We hope that the information provided will be useful to you. Take care of yourself and stay he althy!
