Polycythemia is a chronic disease in which the content of red blood cells (red blood cells) in the blood increases. Also, with this pathology, in 70% of patients, the platelet and white blood cell counts increase.
The disease does not have a high prevalence - no more than five cases are recorded annually per one million people. Most often, the disease polycythemia develops in middle-aged and elderly people. According to statistics, males suffer from this pathology five times more often than women. Today we consider in more detail such a condition as polycythemia, symptoms and treatment of pathology will be described below.
Causes of the disease
Polycythemia is not a malignant disease. To date, the exact causes of the ailment are unknown. It is believed that the development of pathology causes a mutation of a particular bone marrow enzyme. Gene changes lead to excessive division and growth of all blood cells, and especially red blood cells.
Disease classification
There are two groups of the disease:
True polycythemia, 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, elevation).
Relative polycythemia (stressful 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 examination for completely different reasons, true polycythemia can be accidentally detected. Symptoms to which we should pay attention, we consider further.
Saphenous veins
With polycythemia on the skin, most often in the neck, extended saphenous veins appear. With this pathology, the skin becomes a reddish-cherry hue, this is especially noticeable in open areas of the body - neck, hands, face. The mucous membrane of the lips and tongue has a bluish-red color, as if the whites of the eyes were poured with blood.
The overflow of red blood cells rich in red blood cells of all superficial vessels and slowing of its rheological properties (speed of movement) causes such changes, as a result of which the main part of hemoglobin (red pigment) goes into its restored form (i.e. undergoes chemical changes) and changes color.
Itchy skin
In almost half of patients with polycythemia, severe skin itching occurs, especially manifested after taking a warm bath. This phenomenon acts as a specific sign of true polycythemia. Itching occurs due to the release of active substances into the bloodstream, in particular histamine, which is able to dilate skin capillaries, which leads to increased blood circulation in them and the appearance of specific sensations.
Erythromelalagia
This phenomenon is characterized by short-term severe pain in the area of the fingertips. It provokes an increase in the level of platelets in the small vessels of the hand, as a result of which numerous microthrombi form, clogging the arterioles and blocking the flow of blood to the tissues of the fingers. External signs of this condition are redness and the appearance of bluish spots on the skin. In order to prevent thrombosis, it is recommended to take aspirin.
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.
Duodenal and stomach ulcers
Such a serious surgical pathology develops due to thrombosis of small vessels of the mucous membrane of the digestive tract. The result of acute circulatory failure is necrosis (necrosis) of the organ wall section and the formation of a peptic ulcer in its place. In addition, the resistance of the stomach to Helicobacter is reduced (a microorganism that causes gastritis and ulcers).
Blood clots in large vessels
Veins of the lower extremities are more susceptible to this pathology. Blood clots, breaking away from the vessel wall, can bypass the heart, penetrate into the pulmonary circulation (lungs) and provoke pulmonary embolism (pulmonary embolism) - a condition incompatible with life.
Gum bleeding
Despite the fact that the platelet count in the peripheral blood changes and its coagulability increases, with polycythemia gingival bleeding can occur.
Gout
With an increase in the level of uric acid, its salts are deposited in various joints and provoke a sharp pain syndrome.
- Pain in the limbs. Such a symptom causes damage to the arteries of the legs, their narrowing and, as a result, circulatory disorders. This pathology has the name "obliterating endarteritis"
- Pain in flat bones. The increased activity of the bone marrow (the place of development of blood cells) provokes the sensitivity of flat bones to mechanical stress.
Deterioration of the general condition of the body
In a disease such as polycythemia, the symptoms may be similar to signs of other pathologies (for example, anemia): headaches, constant fatigue, tinnitus, dizziness, flickering “goosebumps” in front of the eyes, shortness of breath, flushing of blood to the head. An increase in the viscosity properties of blood activates a compensatory reaction of blood vessels, as a result, there is an increase in blood pressure. With this pathology, complications are often observed in the form of heart failure and microcardiosclerosis (replacing the muscle tissue of the heart with a connective tissue that fills the defect but does not fulfill the necessary functions).
Diagnostics
Polycythemia is revealed by the results of a general blood test, in which it is found:
increased number of red cells from 6.5 to 7.5 • 10 ^ 12 / l;
increased hemoglobin level - up to 240 g / l;
the total volume of red blood cells (OOE) exceeds 52%.
Since the number of red blood cells cannot be calculated based on measurements of the above values, radionuclide diagnostics are used to measure. If the mass of red blood cells exceeds 36 ml / kg in men and 32 ml / kg in women, then this reliably indicates the presence of Wakez disease.
With polycythemia, the morphology of red blood cells 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 is observed (a decrease in red blood cells).
Polycythemia: treatment
A good therapeutic effect has bloodletting. It is recommended that 200-300 ml of blood be removed weekly until the level of OOE drops to the desired value. If there are contraindications for bloodletting, you can restore the percentage of red blood cells by diluting the blood, adding the liquid part to it (high molecular weight solutions are administered intravenously).
It should be borne in mind that quite often bloodletting leads to the development of iron deficiency anemia, in which corresponding symptoms and an increase in the platelet content are observed.
With such an ailment as true polycythemia, treatment involves following a certain diet. It is recommended to limit the use of meat and fish products, as they contain a high amount of protein, which actively stimulates the activity of blood formation organs. Fatty foods should also be discarded. Cholesterol contributes to the development of atherosclerosis, as a result of which there are blood clots, which in large quantities are already formed in people suffering from polycythemia.
With such a disease, it is recommended to give preference to dairy and vegetable products, as well as to limit physical activity.
Also, if polycythemia is diagnosed, treatment may include chemotherapy. Apply it with increased thrombocytosis and severe itching. As a rule, this is a “cytoreductive agent” (the drug “Hydroxycarbamide”).
Until recently, injections of radioactive isotopes (usually phosphorus-32) were used to suppress bone marrow. Today, they increasingly refuse such treatment, due to the high speed of leukemic transformation.
The therapy also includes injections of interferon. Anagrelide is used in the treatment of secondary thrombocytosis.
With this pathology, bone marrow transplantation is very rare , as polycythemia is a disease that is not fatal, provided, of course, adequate treatment and constant monitoring.
Polycythemia in the newborn
Polycythemia is a pathology whose symptoms can be detected in newborns. This disease is a response of the crumb body to previous hypoxia, which placental insufficiency could provoke . The baby's body begins to synthesize a large number of red blood cells to correct hypoxia.
In addition to respiratory conditioning, true polycythemia can also develop in newborns. The twins are especially 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 level.
Neonatal polycythemia has several stages of the course: initial, stage of polyperation and depletion. We will briefly describe them.
The initial stage of the disease has virtually no clinical manifestations. It is possible to detect polycythemia in a child at this stage only by examining the parameters of peripheral blood: hematocrit, hemoglobin, and the level of red blood cells.
An enlargement of the liver and spleen develops at the polyperation stage. Plethoric phenomena are observed: skin integuments acquire a characteristic “pletoric-red” shade, the child is anxious when touching the skin. Pletoric syndrome is supplemented by thrombosis. In the analyzes, there is a change in the number of red blood cells, platelets and leukocyte shifts. The indicators of all blood cells can also increase, this phenomenon is called "panmyelosis".
The stage of exhaustion is characterized by significant loss of body weight, asthenization and exhaustion.
For a newborn, such clinical changes are extremely difficult and can cause irreversible changes and subsequent death. Polycythemia can cause a violation of the production of a certain type of white blood cells, which are responsible for the body's immune system. As a result, the baby develops severe infections of a bacterial nature, resulting in death.
After reading this article, you learned more about a pathology such as polycythemia. Symptoms and treatment were examined by us in as much detail as possible. We hope you find the information provided useful. Take care of yourself and be healthy!