What is dangerous hemolytic anemia. Forms of hemolytic anemia

The erythrocyte membrane consists of a double lipid layer penetrated by various proteins that act as pumps for various microelements. Elements of the cytoskeleton are attached to the inner surface of the membrane. On the outer surface of the erythrocyte is a large number of glycoproteins that act as receptors and antigens - molecules that determine the uniqueness of the cell. To date, more than 250 types of antigens have been found on the surface of erythrocytes, the most studied of which are antigens of the AB0 system and the Rh factor system.

There are 4 blood groups according to the AB0 system, and 2 groups according to the Rh factor. The discovery of these blood groups marked the beginning of a new era in medicine, as it made it possible to transfuse blood and its components to patients with malignant blood diseases, massive blood loss, etc. Also, thanks to blood transfusion, the survival rate of patients after massive surgical interventions has significantly increased.

According to the AB0 system, they distinguish following groups blood:

agglutinogens ( antigens on the surface of red blood cells that, when in contact with the same agglutinins, cause precipitation of red blood cells) are absent on the surface of erythrocytes;
agglutinogens A are present;
agglutinogens B are present;
Agglutinogens A and B are present.

By the presence of the Rh factor, the following blood groups are distinguished:

Rh-positive - 85% of the population;
Rh-negative - 15% of the population.

Despite the fact that, theoretically, transfusing completely compatible blood there should be no anaphylactic reactions from one patient to another, they happen periodically. The reason for this complication is incompatibility for other types of erythrocyte antigens, which, unfortunately, are practically not studied today. In addition, some components of plasma, the liquid part of blood, can be the cause of anaphylaxis. Therefore, according to the latest recommendations of international medical guides, whole blood transfusion is not welcome. Instead, blood components are transfused - red blood cells, platelets, albumins, fresh frozen plasma, clotting factor concentrates, etc.

The previously mentioned glycoproteins, located on the surface of the erythrocyte membrane, form a layer called the glycocalyx. An important feature of this layer is the negative charge on its surface. The surface of the inner layer of vessels also has a negative charge. Accordingly, in the bloodstream, erythrocytes are repelled from the walls of the vessel and from each other, which prevents the formation blood clots. However, as soon as an erythrocyte is damaged or the vessel wall is injured, their negative charge is gradually replaced by a positive one, healthy erythrocytes are grouped around the site of damage, and a thrombus is formed.

The concept of deformability and cytoplasmic viscosity of an erythrocyte is closely related to the functions of the cytoskeleton and the concentration of hemoglobin in the cell. Deformability is the ability of a cell erythrocyte to arbitrarily change its shape to overcome obstacles. Cytoplasmic viscosity is inversely proportional to deformability and increases with an increase in hemoglobin content relative to the liquid part of the cell. The increase in viscosity occurs during aging of the erythrocyte and is a physiological process. In parallel with the increase in viscosity, there is a decrease in deformability.

However, changes in these indicators can take place not only when physiological process aging of the erythrocyte, but also in many congenital and acquired pathologies, such as hereditary membranopathies, fermentopathies and hemoglobinopathies, which will be described in more detail below.

The erythrocyte, like any other living cell, needs energy to function successfully. The erythrocyte receives energy during redox processes occurring in mitochondria. Mitochondria are compared to the powerhouses of the cell as they convert glucose into ATP in a process called glycolysis. A distinctive feature of the erythrocyte is that its mitochondria form ATP only by anaerobic glycolysis. In other words, these cells do not need oxygen to ensure their vital activity and therefore deliver exactly as much oxygen to the tissues as they received when passing through the pulmonary alveoli.

Despite the fact that erythrocytes have been considered as the main carriers of oxygen and carbon dioxide, in addition to this, they perform a number of important functions.

The secondary functions of erythrocytes are:

regulation acid-base balance blood through a carbonate buffer system;
hemostasis - a process aimed at stopping bleeding;
determination of the rheological properties of blood - a change in the number of red blood cells in relation to the total amount of plasma leads to thickening or thinning of the blood.
participation in immune processes - on the surface of the erythrocyte there are receptors for attaching antibodies;
digestive function - decaying, erythrocytes release heme, which independently transforms into free bilirubin. In the liver, free bilirubin is converted into bile, which is used to break down fats in food.

Life cycle of an erythrocyte

Red blood cells are formed in the red bone marrow, passing through numerous stages of growth and maturation. All intermediate forms of erythrocyte precursors are combined into a single term - erythrocyte germ.

As they mature, erythrocyte precursors undergo a change in the acidity of the cytoplasm ( liquid part of the cell), self-digestion of the nucleus and accumulation of hemoglobin. The immediate precursor of the erythrocyte is the reticulocyte - a cell in which, when viewed under a microscope, one can find some dense inclusions that were once the nucleus. Reticulocytes circulate in the blood for 36 to 44 hours, during which they get rid of the remnants of the nucleus and complete the synthesis of hemoglobin from the residual messenger RNA strands ( ribonucleic acid).

The regulation of the maturation of new red blood cells is carried out through a direct feedback mechanism. A substance that stimulates the growth of the number of red blood cells is erythropoietin, a hormone produced by the kidney parenchyma. With oxygen starvation, the production of erythropoietin increases, which leads to an acceleration of the maturation of erythrocytes and, ultimately, the restoration of the optimal level of tissue oxygen saturation. Secondary regulation of the activity of the erythrocyte germ is carried out through interleukin-3, stem cell factor, vitamin B 12, hormones ( thyroxine, somatostatin, androgens, estrogens, corticosteroids) and trace elements ( selenium, iron, zinc, copper, etc.).

After 3-4 months of the existence of an erythrocyte, its gradual involution occurs, which is manifested by the release of intracellular fluid from it due to the wear of most of the transport enzyme systems. This is followed by compaction of the erythrocyte, accompanied by a decrease in its plastic properties. The decrease in plastic properties impairs the permeability of the erythrocyte through the capillaries. Ultimately, such an erythrocyte enters the spleen, gets stuck in its capillaries and is destroyed by leukocytes and macrophages located around them.

After the destruction of the erythrocyte, free hemoglobin is released into the bloodstream. When the rate of hemolysis is less than 10% of total number red blood cells per day, hemoglobin is captured by a protein called haptoglobin and deposited in the spleen and the inner layer of blood vessels, where it is destroyed by macrophages. Macrophages destroy the protein portion of hemoglobin but release heme. Under the action of a number of blood enzymes, heme is transformed into free bilirubin, after which it is transported to the liver by the protein albumin. The presence of a large amount of free bilirubin in the blood is accompanied by the appearance of lemon-colored jaundice. In the liver, free bilirubin binds to glucuronic acid and is excreted in the intestines as bile. If there is an obstruction to the outflow of bile, it enters the bloodstream and circulates in the form bound bilirubin. In this case, jaundice also appears, but of a darker shade ( mucous membranes and skin are orange or reddish in color).

After the release of conjugated bilirubin into the intestine in the form of bile, it is reduced to stercobilinogen and urobilinogen with the help of intestinal flora. Most of the stercobilinogen is converted to stercobilin, which is excreted in the feces and turns it brown. The rest of the stercobilinogen and urobilinogen are absorbed in the intestine and returned to the bloodstream. Urobilinogen is converted to urobilin and excreted in the urine, while stercobilinogen is re-entered by the liver and excreted in the bile. This cycle at first glance may seem meaningless, however, this is a delusion. During the re-entry of the decay products of red blood cells into the blood, the activity of the immune system is stimulated.

With an increase in the rate of hemolysis from 10% to 17-18% of the total number of erythrocytes per day, haptoglobin reserves become insufficient to capture the released hemoglobin and utilize it in the way described above. In this case, free hemoglobin with the blood flow enters the renal capillaries, is filtered into the primary urine and oxidized to hemosiderin. Then hemosiderin enters the secondary urine and is excreted from the body.

With extremely pronounced hemolysis, the rate of which exceeds 17 - 18% of the total number of red blood cells per day, hemoglobin enters the kidneys in too much quantity. Because of this, its oxidation does not have time to occur and pure hemoglobin enters the urine. Thus, the determination of excess urobilin in the urine is a sign of mild hemolytic anemia. The appearance of hemosiderin indicates a transition to middle degree hemolysis. The detection of hemoglobin in the urine indicates a high intensity of destruction of red blood cells.

What is hemolytic anemia?

Hemolytic anemia is a disease in which the duration of the existence of erythrocytes is significantly shortened due to a number of external and internal erythrocyte factors. Internal factors leading to the destruction of erythrocytes are various anomalies in the structure of erythrocyte enzymes, heme or cell membrane. External factors that can lead to the destruction of an erythrocyte are various kinds of immune conflicts, mechanical failure erythrocytes, as well as infection of the body with certain infectious diseases.

Hemolytic anemias are classified into congenital and acquired.

There are the following types of congenital hemolytic anemia:

membranopathies;
fermentopathy;
hemoglobinopathies.

There are the following types of acquired hemolytic anemia:

immune hemolytic anemia;
acquired membranopathies;
anemia due to mechanical destruction of red blood cells;
hemolytic anemia caused by infectious agents.

Congenital hemolytic anemias

Membranopathy

As previously described, normal form erythrocyte is the shape of a biconcave disc. This shape corresponds to the correct protein composition of the membrane and allows the erythrocyte to penetrate through capillaries, the diameter of which is several times smaller than the diameter of the erythrocyte itself. The high penetrating ability of erythrocytes, on the one hand, allows them to most effectively perform their main function - the exchange of gases between the internal environment of the body and external environment, and on the other hand, to avoid their excessive destruction in the spleen.

A defect in certain membrane proteins leads to a violation of its shape. With a violation of the form, there is a decrease in the deformability of erythrocytes and, as a result, their increased destruction in the spleen.

To date, there are 3 types of congenital membranopathies:

microspherocytosis
ovalocytosis

Acanthocytosis called a condition in which erythrocytes with numerous outgrowths, called acanthocytes, appear in the patient's bloodstream. The membrane of such erythrocytes is not rounded and resembles an edge under a microscope, hence the name of the pathology. The causes of acanthocytosis are not fully understood to date, however, there is a clear relationship between this pathology and severe liver damage with high blood fat values ( total cholesterol and its fractions, beta-lipoproteins, triacylglycerides, etc.). A combination of these factors may occur in hereditary diseases such as Huntington's chorea and abetalipoproteinemia. The acanthocytes are unable to pass through the capillaries of the spleen and therefore are soon destroyed, leading to hemolytic anemia. Thus, the severity of acanthocytosis directly correlates with the intensity of hemolysis and clinical signs anemia.

microspherocytosis- a disease that in the past was called familial hemolytic jaundice, since it has a clear autosomal recessive inheritance of a defective gene responsible for the formation of a biconcave form of an erythrocyte. As a result, in such patients, all formed erythrocytes differ in a spherical shape and a smaller diameter, in relation to healthy red blood cells. spherical shape has a smaller surface area compared to the normal biconcave shape, so the efficiency of gas exchange of such erythrocytes is reduced. Moreover, they contain a smaller amount of hemoglobin and change worse when passing through the capillaries. These features lead to a shortening of the lifespan of such red blood cells through premature hemolysis in the spleen.

Since childhood, such patients have hypertrophy of the erythrocyte bone marrow germ, compensating for hemolysis. Therefore, with microspherocytosis, mild and moderate anemia is more often observed, which appears mainly at moments of weakening of the body. viral diseases, malnutrition or intense physical labor.

Ovalocytosis is a hereditary disease transmitted in an autosomal dominant manner. More often the disease proceeds subclinically with the presence in the blood of less than 25% of oval erythrocytes. Much less common severe forms, at which the number of defective erythrocytes approaches 100%. The cause of ovalocytosis lies in a defect in the gene responsible for the synthesis of the spectrin protein. Spectrin is involved in the construction of the erythrocyte cytoskeleton. Thus, due to insufficient plasticity of the cytoskeleton, the erythrocyte is not able to restore its biconcave shape after passing through the capillaries and circulates in peripheral blood in the form of ellipsoidal cells. The more pronounced the ratio of the longitudinal and transverse diameter of the ovalocyte, the sooner its destruction occurs in the spleen. Removal of the spleen significantly reduces the rate of hemolysis and leads to remission of the disease in 87% of cases.

Fermentopathies

The erythrocyte contains a number of enzymes that maintain the constancy of its internal environment, process glucose into ATP and regulate the acid-base balance of the blood.

According to the above directions, there are 3 types of fermentopathy:

deficiency of enzymes involved in the oxidation and reduction of glutathione ( see below);
deficiency of glycolysis enzymes;
deficiency of enzymes that use ATP.

Glutathione is a tripeptide complex involved in most redox processes in the body. In particular, it is necessary for the work of mitochondria - the energy stations of any cell, including the erythrocyte. Congenital defects in enzymes involved in the oxidation and reduction of erythrocyte glutathione lead to a decrease in the rate of production of ATP molecules, the main energy substrate for most energy-dependent cell systems. ATP deficiency leads to a slowdown in the metabolism of red blood cells and their rapid self-destruction, called apoptosis.

glycolysis is the process of breakdown of glucose with the formation of ATP molecules. Glycolysis requires the presence of a number of enzymes that repeatedly convert glucose into intermediates and eventually release ATP. As stated earlier, an erythrocyte is a cell that does not use oxygen to form ATP molecules. This type of glycolysis is anaerobic ( airless). As a result, 2 ATP molecules are formed from one glucose molecule in an erythrocyte, which are used to maintain the efficiency of most of the cell's enzyme systems. Accordingly, a congenital defect in glycolysis enzymes deprives the erythrocyte required amount energy to sustain life, and it collapses.

ATP is a universal molecule, the oxidation of which releases the energy necessary for the operation of more than 90% of the enzyme systems of all body cells. The erythrocyte also contains many enzyme systems, the substrate of which is ATP. The released energy is spent on the process of gas exchange, maintaining a constant ionic balance inside and outside the cell, maintaining a constant osmotic and oncotic pressure of the cell, as well as on the active work of the cytoskeleton, and much more. Violation of glucose utilization in at least one of the above systems leads to a loss of its function and a further chain reaction, the result of which is the destruction of the erythrocyte.

Hemoglobinopathies

Hemoglobin is a molecule that occupies 98% of the volume of an erythrocyte, responsible for ensuring the processes of capturing and releasing gases, as well as for their transportation from the pulmonary alveoli to peripheral tissues and vice versa. With some defects in hemoglobin, erythrocytes carry gases much worse. In addition, against the background of a change in the hemoglobin molecule, the shape of the erythrocyte itself also changes, which also negatively affects the duration of their circulation in the bloodstream.

There are 2 types of hemoglobinopathies:

quantitative - thalassemia;
qualitative - sickle cell anemia or drepanocytosis.

Thalassemia are hereditary diseases associated with impaired hemoglobin synthesis. By its structure, hemoglobin is a complex molecule consisting of two alpha monomers and two beta monomers linked together. The alpha chain is synthesized from 4 sections of DNA. Beta chain - from 2 sections. Thus, when a mutation occurs in one of the 6 regions, the synthesis of the monomer whose gene is damaged decreases or stops. Healthy genes continue to synthesize monomers, which over time leads to the quantitative predominance of some chains over others. Those monomers that are in excess form fragile compounds, the function of which is much inferior to normal hemoglobin. According to the chain, the synthesis of which is impaired, there are 3 main types of thalassemia - alpha, beta and mixed alpha-beta thalassemia. The clinical picture depends on the number of mutated genes.

sickle cell anemia is a hereditary disease in which abnormal hemoglobin S is formed instead of normal hemoglobin A. This abnormal hemoglobin is significantly inferior in functionality to hemoglobin A, and also changes the shape of the red blood cell to crescent. This form leads to the destruction of erythrocytes in a period of 5 to 70 days in comparison with normal duration their existence is from 90 to 120 days. As a result, a proportion of sickle-shaped erythrocytes appears in the blood, the value of which depends on whether the mutation is heterozygous or homozygous. With a heterozygous mutation, the proportion of abnormal erythrocytes rarely reaches 50%, and the patient experiences symptoms of anemia only with significant physical exertion or in conditions of reduced oxygen concentration in the atmospheric air. With a homozygous mutation, all the patient's erythrocytes are sickle-shaped, and therefore the symptoms of anemia appear from the birth of the child, and the disease is characterized by a severe course.

Acquired hemolytic anemia

Immune hemolytic anemias

With this type of anemia, the destruction of red blood cells occurs under the influence of the body's immune system.

There are 4 types of immune hemolytic anemias:

autoimmune;
isoimmune;
heteroimmune;
transimmune.

With autoimmune anemia the patient's own body produces antibodies to normal red blood cells due to a malfunction of the immune system and a violation of the recognition of own and foreign cells by lymphocytes.

Isoimmune anemia develop when a patient is transfused with blood that is incompatible in terms of the AB0 system and the Rh factor, or, in other words, blood of another group. In this case, the day before, the transfused red blood cells are destroyed by the cells of the immune system and the antibodies of the recipient. A similar immune conflict develops with positive Rh factor in the blood of the fetus and negative - in the blood of the pregnant mother. This pathology is called hemolytic disease of newborns.

Heteroimmune anemias develop when foreign antigens appear on the erythrocyte membrane, which are recognized by the patient's immune system as foreign. Foreign antigens may appear on the surface of the erythrocyte in the case of the use of certain medications or after acute viral infections.

Transimmune anemias develop in the fetus when antibodies against red blood cells are present in the mother's body ( autoimmune anemia). In this case, both maternal and fetal erythrocytes become the target of the immune system, even if Rh incompatibility is not detected, as in hemolytic disease newborns.

Acquired membranopathies

A representative of this group is paroxysmal nocturnal hemoglobinuria or Marchiafava-Micheli disease. At the core this disease there is a constant formation of a small percentage of red blood cells with a defective membrane. Presumably an erythrocyte germ of a certain area bone marrow undergoes a mutation caused by various harmful factors, such as radiation, chemical agents, etc. The resulting defect makes red blood cells unstable to contact with complement system proteins ( one of the main components of the body's immune defense). Thus, healthy erythrocytes are not deformed, and defective erythrocytes are destroyed by complement in the bloodstream. As a result, a large amount of free hemoglobin is released, which is excreted in the urine mainly at night.

Anemia due to mechanical destruction of red blood cells

This group of diseases includes:

marching hemoglobinuria;
microangiopathic hemolytic anemia;
anemia in mechanical heart valve transplants.

Marching hemoglobinuria, based on the name, develops during long marching. The formed elements of the blood located in the feet, with prolonged regular compression of the soles, are deformed and even destroyed. As a result, a large amount of unbound hemoglobin is released into the blood, which is excreted in the urine.

Microangiopathic hemolytic anemia develops due to deformation and subsequent destruction of erythrocytes in acute glomerulonephritis and disseminated intravascular coagulation syndrome. In the first case, due to inflammation of the renal tubules and, accordingly, the capillaries surrounding them, their lumen narrows, and the erythrocytes are deformed by friction with their inner membrane. In the second case, throughout circulatory system lightning-fast platelet aggregation occurs, accompanied by the formation of many fibrin filaments that block the lumen of the vessels. Part of the erythrocytes immediately gets stuck in the formed network and forms multiple blood clots, and the remaining part slips through this network at high speed, deforming along the way. As a result, red blood cells deformed in this way, called "crowned", still circulate in the blood for some time, and then are destroyed on their own or when passing through the capillaries of the spleen.

Anemia in Mechanical Heart Valve Transplant develops when red blood cells moving at high speed collide with the dense plastic or metal that makes up an artificial heart valve. The rate of destruction depends on the rate of blood flow in the area of the valve. Hemolysis increases with physical work, emotional experiences, a sharp increase or decrease in blood pressure and an increase in body temperature.

Hemolytic anemia caused by infectious agents

Microorganisms such as Plasmodium malaria and Toxoplasma gondii ( causative agent of toxoplasmosis) use erythrocytes as a substrate for reproduction and growth of their own kind. As a result of infection with these infections, pathogens penetrate the erythrocyte and multiply in it. Then, after a certain time, the number of microorganisms increases so much that it destroys the cell from the inside. At the same time, an even greater amount of the pathogen is released into the blood, which is populated in healthy red blood cells and repeats the cycle. As a result, in malaria every 3 to 4 days ( depending on the type of pathogen) there is a wave of hemolysis, accompanied by a rise in temperature. With toxoplasmosis, hemolysis develops according to a similar scenario, but more often it has a non-wave course.

Causes of hemolytic anemia

Summarizing all the information from the previous section, it is safe to say that there are a lot of causes of hemolysis. The reasons can lie both in hereditary diseases and in acquired ones. It is for this reason that great importance is attached to the search for the cause of hemolysis not only in the blood system, but also in other body systems, since the destruction of red blood cells is often not an independent disease, but a symptom of another disease.

Thus, hemolytic anemia can develop for the following reasons:

entry into the blood of various toxins and poisons ( pesticides, pesticides, snake bites, etc.);
mechanical destruction of erythrocytes ( during many hours of walking, after implantation artificial valve hearts, etc.);
disseminated intravascular coagulation syndrome;
various genetic abnormalities structure of erythrocytes;
autoimmune diseases;
paraneoplastic syndrome ( cross-immunodestruction of erythrocytes along with tumor cells );
complications after transfusion donated blood;
infection with some infectious diseases ( malaria, toxoplasmosis);
chronic glomerulonephritis;
severe purulent infections accompanied by sepsis;
infectious hepatitis B, less often C and D;
avitaminosis, etc.

Symptoms of hemolytic anemia

Symptoms of hemolytic anemia fit into two main syndromes - anemic and hemolytic. In the case when hemolysis is a symptom of another disease, the clinical picture is complicated by its symptoms.

Anemia syndrome is manifested by the following symptoms:

pallor of the skin and mucous membranes;
dizziness;
severe general weakness;
fast fatigue;
shortness of breath during normal physical activity;
heartbeat;

Hemolytic syndrome is manifested by the following symptoms:

icteric-pale color of the skin and mucous membranes;
dark brown, cherry, or scarlet urine;
an increase in the size of the spleen;
pain in the left hypochondrium, etc.

Diagnosis of hemolytic anemia

Diagnosis of hemolytic anemia is carried out in two stages. At the first stage, hemolysis occurring in vascular bed or in the spleen. At the second stage, numerous additional studies are carried out to determine the cause of the destruction of red blood cells.

First stage of diagnosis

Hemolysis of erythrocytes is of two types. The first type of hemolysis is called intracellular, that is, the destruction of red blood cells occurs in the spleen through the absorption of defective red blood cells by lymphocytes and phagocytes. The second type of hemolysis is called intravascular, that is, the destruction of red blood cells takes place in the bloodstream under the action of lymphocytes, antibodies and complement circulating in the blood. Determining the type of hemolysis is extremely important, because it gives the researcher a hint in which direction to continue searching for the cause of the destruction of red blood cells.

Confirmation of intracellular hemolysis is carried out using the following laboratory parameters:

hemoglobinemia- the presence of free hemoglobin in the blood due to the active destruction of red blood cells;
hemosiderinuria- the presence in the urine of hemosiderin - a product of oxidation in the kidneys of excess hemoglobin;
hemoglobinuria- the presence in the urine of unchanged hemoglobin, a sign of an extremely high rate of destruction of red blood cells.

Confirmation of intravascular hemolysis is carried out using the following laboratory tests:

complete blood count - a decrease in the number of red blood cells and / or hemoglobin, an increase in the number of reticulocytes;
biochemical blood test - an increase in total bilirubin due to the indirect fraction.
peripheral blood smear - with various methods of staining and fixing the smear, most of the anomalies in the structure of the erythrocyte are determined.

When hemolysis is ruled out, the researcher switches to the search for another cause of anemia.

The second stage of diagnosis

There are a lot of reasons for the development of hemolysis, so their search can take an unacceptably long time. In this case, it is necessary to clarify the history of the disease in as much detail as possible. In other words, it is required to find out the places that the patient visited in the last six months, where he worked, in what conditions he lived, the order in which the symptoms of the disease appeared, the intensity of their development, and much more. Such information may be useful in narrowing the search for the causes of hemolysis. In the absence of such information, a number of analyzes are carried out to determine the substrate most frequent illnesses leading to the destruction of erythrocytes.

Analyzes of the second stage of diagnostics are:

direct and indirect test Coombs;
circulating immune complexes;
osmotic resistance of erythrocytes;
study of the activity of erythrocyte enzymes ( glucose-6-phosphate dehydrognase (G-6-PDH), pyruvate kinase, etc.);
hemoglobin electrophoresis;
erythrocyte crescent test;
test for Heinz bodies;
bacteriological blood culture;
study of a "thick drop" of blood;
myelogram;
Hem's test, Hartman's test ( sucrose test).

Direct and indirect Coombs test
These tests are performed to confirm or rule out autoimmune hemolytic anemia. Circulating immune complexes indirectly indicate the autoimmune nature of hemolysis.

Osmotic resistance of erythrocytes
A decrease in the osmotic resistance of erythrocytes often develops in congenital forms of hemolytic anemia, such as spherocytosis, ovalocytosis, and acanthocytosis. In thalassemia, on the contrary, there is an increase in the osmotic resistance of erythrocytes.

Study of the activity of erythrocyte enzymes
For this purpose, first, qualitative analyzes are carried out for the presence or absence of the desired enzymes, and then they resort to quantitative analyzes carried out using PCR ( polymerase chain reaction) . Quantitative determination of erythrocyte enzymes makes it possible to detect their decrease in relation to normal values and to diagnose latent forms of erythrocyte fermentopathy.

Hemoglobin electrophoresis
The study is carried out in order to exclude both qualitative and quantitative hemoglobinopathies ( thalassemia and sickle cell anemia).

RBC crescent test
The essence of this study is to determine the change in the shape of erythrocytes as the partial pressure of oxygen in the blood decreases. If the red blood cells take on a crescent shape, then the diagnosis of sickle cell anemia is considered confirmed.

Heinz body test
The purpose of this test is to detect special inclusions in a blood smear, which are insoluble hemoglobin. This test is carried out to confirm such fermentopathy as G-6-PDG deficiency. However, it must be remembered that Heinz bodies can appear in a blood smear with an overdose of sulfonamides or aniline dyes. The definition of these formations is carried out in a dark-field microscope or in a conventional light microscope with special staining.

Bacteriological blood culture
Tank culture is performed to determine the types of infectious agents circulating in the blood that can interact with erythrocytes and cause their destruction either directly or through immune mechanisms.

Study of the "thick drop" of blood
This study carried out to identify causative agents of malaria, life cycle which is closely associated with the destruction of erythrocytes.

Myelogram
Myelogram is the result of a bone marrow puncture. This paraclinical method allows to identify such pathologies as malignant diseases blood, which, through a cross-immune attack in paraneoplastic syndrome, also destroy red blood cells. In addition, proliferation of erythroid germ is determined in the bone marrow punctate, which indicates a high rate of compensatory production of erythrocytes in response to hemolysis.

Ham test. Hartman's test ( sucrose test)
Both tests are carried out in order to determine the duration of the existence of erythrocytes of a particular patient. In order to speed up the process of their destruction, the tested blood sample is placed in weak solution acids or sucrose, and then estimate the percentage of destroyed red blood cells. Hem's test is considered positive when more than 5% of red blood cells are destroyed. The Hartman test is considered positive when more than 4% of red blood cells are destroyed. A positive test indicates paroxysmal nocturnal hemoglobinuria.

In addition to the presented laboratory tests to establish the cause of hemolytic anemia, other additional tests and instrumental studies may be performed, prescribed by a specialist in the field of the disease that is suspected to be the cause of hemolysis.

Treatment of hemolytic anemia

Treatment of hemolytic anemia is a complex multilevel dynamic process. It is preferable to start treatment after a full diagnosis and establishment of the true cause of hemolysis. However, in some cases, the destruction of red blood cells occurs so quickly that there is not enough time to establish a diagnosis. In such cases, as a forced measure, the lost erythrocytes are replenished by transfusion of donor blood or washed erythrocytes.

Treatment of primary idiopathic ( unclear reason ) hemolytic anemia, as well as secondary hemolytic anemia due to diseases of the blood system, is dealt with by a hematologist. Treatment of secondary hemolytic anemia due to other diseases falls to the lot of the specialist in whose field of activity this disease is located. Thus, anemia caused by malaria will be treated by an infectious disease doctor. Autoimmune anemia will be treated by an immunologist or an allergist. Anemia due to paraneoplastic syndrome in a malignant tumor will be treated by an oncosurgeon, etc.

Treatment of hemolytic anemia with medicines

The basis of the treatment of autoimmune diseases and, in particular, hemolytic anemia are glucocorticoid hormones. They are used for a long time - first to stop the exacerbation of hemolysis, and then as a maintenance treatment. Since glucocorticoids have a number of side effects, then for their prevention, auxiliary treatment with B vitamins and drugs that reduce the acidity of gastric juice is carried out.

In addition to reducing autoimmune activity, much attention should be paid to the prevention of DIC ( blood clotting disorder), especially at medium and high intensity of hemolysis. With low efficacy of glucocorticoid therapy, immunosuppressants are the last line of treatment.

Medication	Mechanism of action	Mode of application
*Prednisolone*	It is a representative of glucocorticoid hormones, which have the most pronounced anti-inflammatory and immunosuppressive effects.	1 - 2 mg / kg / day intravenously, drip. With severe hemolysis, the dose of the drug is increased to 150 mg / day. After normalization of hemoglobin levels, the dose is slowly reduced to 15-20 mg / day and treatment is continued for another 3-4 months. After that, the dose is reduced by 5 mg every 2 to 3 days until the drug is completely discontinued.
*Heparin*	It is a short acting direct anticoagulant 4 – 6 hours). This drug is prescribed for the prevention of DIC, which often develops with acute hemolysis. It is used in the unstable condition of the patient for better control of coagulation.	2500 - 5000 IU subcutaneously every 6 hours under the control of a coagulogram.
*Nadroparin*	Is a direct anticoagulant long-acting (24 – 48 hours). It is prescribed to patients with a stable condition for the prevention of thromboembolic complications and DIC.	0.3 ml / day subcutaneously under the control of a coagulogram.
*Pentoxifylline*	Peripheral vasodilator with moderate antiplatelet action. Increases oxygen supply to peripheral tissues.	400 - 600 mg / day in 2 - 3 oral doses for a minimum of 2 weeks. The recommended duration of treatment is 1-3 months.
*Folic acid*	Belongs to the group of vitamins. In autoimmune hemolytic anemia, it is used to replenish its reserves in the body.	Treatment begins with a dose of 1 mg / day, and then increase it until a stable clinical effect appears. Maximum daily dose- 5 mg.
*Vitamin B 12*	In chronic hemolysis, the reserves of vitamin B 12 are gradually depleted, which leads to an increase in the diameter of the erythrocyte and a decrease in its plastic properties. In order to avoid these complications, an additional appointment of this drug is carried out.	100 - 200 mcg / day intramuscularly.
*Ranitidine*	It is prescribed to reduce the aggressive effect of prednisolone on the gastric mucosa by reducing the acidity of gastric juice.	300 mg / day in 1 - 2 oral doses.
*Potassium chloride*	It is an external source of potassium ions, which are washed out of the body during treatment with glucocorticoids.	2 - 3 g per day under the daily control of the ionogram.
*Cyclosporin A*	A drug from the group of immunosuppressants. It is used as the last line of treatment for the ineffectiveness of glucocorticoids and splenectomy.	3 mg / kg / day intravenously, drip. When expressed side effects the drug is withdrawn with the transition to another immunosuppressant.
*Azathioprine*	Immunosuppressant.
*Cyclophosphamide*	Immunosuppressant.	100 - 200 mg / day for 2 - 3 weeks.
*Vincristine*	Immunosuppressant.	1 - 2 mg / week drip for 3 - 4 weeks.

With a deficiency of G-6-PDG, it is recommended to avoid the use of drugs that are at risk. However, with the development of acute hemolysis against the background of this disease, the drug that caused the destruction of erythrocytes is immediately canceled, and, if necessary, the washed donor erythrocyte mass is transfused.

In severe forms of sickle cell anemia or thalassemia requiring frequent transfusions blood, Deferoxamine is prescribed - a drug that binds excess iron and removes it from the body. Thus, hemochromatosis is prevented. Another option for patients with severe hemoglobinopathies is bone marrow transplantation from a compatible donor. If this procedure is successful, there is a possibility significant improvement the general condition of the patient, until complete recovery.

In the case when hemolysis acts as a complication of a certain systemic disease and is secondary, all therapeutic measures should be aimed at curing the disease that caused the destruction of red blood cells. After the cure primary disease the destruction of erythrocytes also stops.

Surgery for hemolytic anemia

In hemolytic anemia, the most common operation is splenectomy ( splenectomy). This operation indicated for the first recurrence of hemolysis after treatment with glucocorticoid hormones of autoimmune hemolytic anemia. In addition, splenectomy is the preferred treatment for hereditary forms of hemolytic anemia such as spherocytosis, acanthocytosis, and ovalocytosis. Optimal age at which removal of the spleen is recommended in the case of the above diseases is the age of 4-5 years, however, in individual cases, the operation can be performed at an earlier age.

Thalassemia and sickle cell anemia can be treated for a long time by transfusion of washed donor erythrocytes, however, if there are signs of hypersplenism, accompanied by a decrease in the number of other cellular elements in the blood, an operation to remove the spleen is justified.

Prevention of hemolytic anemia

Prevention of hemolytic anemia is divided into primary and secondary. Primary prevention involves measures that prevent the occurrence of hemolytic anemia, and secondary prevention involves reducing the clinical manifestations of an existing disease.

Primary prevention of idiopathic autoimmune anemia is not performed due to the absence of reasons for such.

Primary prevention of secondary autoimmune anemia is:

avoiding associated infections;
avoidance of being in a low temperature environment in anemia with cold antibodies and with high temperature with anemia with warm antibodies;
avoiding snake bites and being in an environment with a high content of toxins and salts of heavy metals;
avoiding the use of medicines from the list below for deficiency of the enzyme G-6-PD.

With a deficiency of G-6-PDH, the following medications cause hemolysis:

antimalarials- primaquine, pamaquine, pentaquine;
painkillers and antipyretics - acetylsalicylic acid (aspirin);
sulfonamides- sulfapyridine, sulfamethoxazole, sulfacetamide, dapsone;
other antibacterial drugs- chloramphenicol, nalidixic acid, ciprofloxacin, nitrofurans;
anti-tuberculosis drugs- ethambutol, isoniazid, rifampicin;
drugs of other groups- probenecid, methylene blue, ascorbic acid, vitamin K analogues.

Secondary prevention consists in the timely diagnosis and appropriate treatment of infectious diseases that can cause an exacerbation of hemolytic anemia.

Hemolytic anemia is a complex of diseases that are combined into one group due to the fact that with all of them the life expectancy of red blood cells decreases. This contributes to the loss of hemoglobin and leads to hemolysis. These pathologies are similar to each other, but their origin, course, and even clinical manifestations differ. Hemolytic anemia in children also has its own characteristics.

Hemolysis is the mass destruction of blood cells. At its core, this is a pathological process that can occur in two spaces of the body.

Extravascular, that is, outside the blood vessels. Most often, the foci are parenchymal organs - the liver, kidneys, spleen, as well as red bone marrow. This type of hemolysis proceeds similarly to physiological;
Intravascular, when blood cells are destroyed in the lumen blood vessels.

Mass destruction of erythrocytes proceeds with a typical symptom complex, while the manifestations of intravascular and extravascular hemolysis are different. They are determined by general examination a patient, a complete blood count and other specific tests will help establish the diagnosis.

Why does hemolysis occur?

The non-physiological death of red blood cells occurs for various reasons, among which iron deficiency in the body occupies one of the most important places. However, this condition should be distinguished from violations of the synthesis of erythrocytes and hemoglobin, which is helped by laboratory tests and clinical symptoms.

Yellowness of the skin, which is displayed by an increase in total bilirubin and its free fraction.
A somewhat distant manifestation is the increased viscosity and density of bile with an increased tendency to stone formation. It also changes color as the content of bile pigments increases. This process is due to the fact that the liver cells are trying to neutralize the excess bilirubin.
The stool also changes its color, as the bile pigments "get" to it, provoking an increase in the levels of stercobilin, urobilinogen.
With extravascular death of blood cells, the level of urobilin rises, which is indicated by darkening of the urine.
A general blood test reacts with a decrease in red blood cells, a drop in hemoglobin. Compensatory growth of young forms of cells - reticulocytes.

Types of erythrocyte hemolysis

The destruction of erythrocytes occurs either in the lumen of blood vessels or in parenchymal organs. Since extravascular hemolysis is similar in its pathophysiological mechanism to the normal death of erythrocytes in parenchymal organs, the difference lies only in its speed, and it is partially described above.

With the destruction of erythrocytes inside the lumen of the vessels develop:

an increase in free hemoglobin, the blood acquires a so-called varnish shade;
discoloration of urine due to free hemoglobin or hemosiderin;
hemosiderosis is a condition when iron-containing pigment is deposited in parenchymal organs.

What is hemolytic anemia

At its core, hemolytic anemia is a pathology in which the lifespan of red blood cells is significantly reduced. This is due big amount factors, either external or internal. Hemoglobin during the destruction of formed elements is partially destroyed, and partially acquires a free form. A decrease in hemoglobin less than 110 g/l indicates the development of anemia. Very rarely, hemolytic anemia is associated with a decrease in the amount of iron.

Internal factors contributing to the development of the disease are anomalies in the structure of blood cells, and external factors are immune conflicts, infectious agents, and mechanical damage.

Classification

The disease can be congenital or acquired, while the development of hemolytic anemia after the birth of a child is called acquired.

Congenital is divided into membranopathies, fermentopathy and hemoglobinopathies, and acquired into immune, acquired membranopathies, mechanical damage to formed elements, due to infectious processes.

To date, doctors do not divide the form of hemolytic anemia at the site of destruction of red blood cells. The most common is autoimmune. Also, most of all fixed pathologies This group accounts for acquired hemolytic anemias, while they are characteristic of all ages, starting from the first months of life. In children, special care should be taken, as these processes may be hereditary. Their development is due to several mechanisms.

The appearance of anti-erythrocyte antibodies that come from outside. In hemolytic disease of the newborn, we are talking about isoimmune processes.
Somatic mutations, which is one of the triggers of chronic hemolytic anemia. It cannot become a genetic hereditary factor.
Mechanical damage to erythrocytes occurs as a result of exposure to heavy physical exertion or prosthetic heart valves.
hypovitaminosis, special role plays vitamin E.
Malarial Plasmodium.
Exposure to poisonous substances.

Autoimmune hemolytic anemia

In autoimmune anemia, the body responds with increased susceptibility to any foreign proteins, and also has an increased tendency to allergic reactions. This is due to an increase in the activity of their own immune system. may change in the blood the following indicators: specific immunoglobulins, the number of basophils and eosinophils.

Autoimmune anemias are characterized by the production of antibodies to normal blood cells, which leads to a violation of the recognition of their own cells. A subspecies of this pathology is transimmune anemia, in which the maternal organism becomes the target of the fetal immune system.

Coombs tests are used to detect the process. They allow you to identify circulating immune complexes that are not present in full health. Allergist or immunologist is engaged in treatment.

Causes

The disease develops for a number of reasons, they can also be congenital or acquired. Approximately 50% of cases of the disease remain without a clarified cause, this form is called idiopathic. Among the causes of hemolytic anemia, it is important to single out those that provoke the process more often than others, namely:

Under the influence of the above triggers and the presence of other triggers, shaped cells are destroyed, contributing to the appearance of symptoms typical of anemia.

Symptoms

Clinical manifestations of hemolytic anemia are quite extensive, but their nature always depends on the cause that caused the disease, one or another of its types. Sometimes the pathology manifests itself only when a crisis or exacerbation develops, and the remission is asymptomatic, the person does not make any complaints.

All the symptoms of the process can be detected only when the state is decompensated, when there is a pronounced imbalance between healthy, emerging and destroyed shaped elements blood, and the bone marrow can not cope with the load placed on it.

Classical clinical manifestations are represented by three symptom complexes:

anemic;
icteric;
enlargement of the liver and spleen - hepatosplenomegaly.

They usually develop with extravascular destruction of formed elements.

Sickle cell, autoimmune and other hemolytic anemias are manifested by such characteristic signs.

Increased body temperature, dizziness. Occurs when the disease progresses rapidly childhood, and the temperature itself reaches 38C.
jaundice syndrome. The appearance of this symptom is due to the destruction of red blood cells, which leads to an increase in the level of indirect bilirubin, which is processed by the liver. His high concentration promotes the growth of stercobilin and intestinal urobilin, due to which feces, skin, and mucous membranes are stained.
As jaundice develops, splenomegaly also develops. This syndrome often occurs with hepatomegaly, that is, both the liver and the spleen are enlarged at the same time.
Anemia. Accompanied by a decrease in the amount of hemoglobin in the blood.

Other signs of hemolytic anemia are:

pain in the epigastrium, abdomen, lumbar region, kidneys, bones;
heart attack-like pain;
malformations of children, accompanied by signs of impaired intrauterine formation of the fetus;
change in the nature of the stool.

Diagnostic methods

Diagnosis of hemolytic anemia is carried out by a hematologist. He establishes the diagnosis on the basis of data obtained during the examination of the patient. First, anamnestic data is collected, the presence of trigger factors is clarified. The doctor assesses the degree of pallor of the skin and visible mucous membranes, conducts a palpation examination of the abdominal organs, in which it is possible to determine an increase in the liver and spleen.

The next step is laboratory and instrumental examination. A general analysis of urine, blood, a biochemical examination is carried out, in which it is possible to establish the presence in the blood high level indirect bilirubin. An ultrasound of the abdominal organs is also performed.

In especially severe cases, a bone marrow biopsy is prescribed, in which it is possible to determine how red blood cells develop in hemolytic anemia. It is important to do the right differential diagnosis to exclude such pathologies as viral hepatitis, hemoblastoses, oncological processes, cirrhosis of the liver, obstructive jaundice.

Treatment

Each individual form of the disease requires its own approach to treatment due to the characteristics of the occurrence. It is important to immediately eliminate all hemolyzing factors, if we are talking about an acquired process. If the treatment of hemolytic anemia occurs during a crisis, then the patient should receive a large amount of blood transfusions - blood plasma, erythrocyte mass, also carry out metabolic and vitamin therapy, with a special role played by compensation for vitamin E deficiency.

Sometimes there is a need to prescribe hormones and antibiotics. If microspherocytosis is diagnosed, the only treatment option is splenectomy.

Autoimmune processes involve the use steroid hormones. Prednisone is considered the drug of choice. Such therapy reduces hemolysis, and sometimes stops it completely. Particularly severe cases require the appointment of immunosuppressants. If the disease is completely resistant to medical drugs, doctors resort to removing the spleen.

In the toxic form of the disease, there is a need for detoxification intensive care- hemodialysis, treatment with antidotes, forced diuresis with preserved kidney function.

Treatment of hemolytic anemia in children

As mentioned earlier, hemolytic anemia is a group pathological processes, which in its mechanism of development can differ significantly, but all diseases have one thing in common - hemolysis. It occurs not only in the bloodstream, but also in parenchymal organs.

The first signs of the development of the process often do not cause any suspicion in sick people. If a child develops anemia rapidly, then irritability, fatigue, tearfulness, and pallor of the skin appear. These signs can be easily mistaken for the characteristics of the character of the baby. Especially when it comes to frequently ill children. And this is not surprising, since in the presence of this pathology, people are prone to the development of infectious processes.

The main symptoms of anemia in children are pallor of the skin, which must be differentiated from renal pathologies, tuberculosis, intoxication of various origins.

The main sign that will allow you to determine the presence of anemia without determining laboratory parameters - with anemia, the mucous membranes also become pale.

Complications and prognosis

The main complications of hemolytic anemia are:

the worst thing is an anemic coma and death;
decrease in blood pressure, accompanied by a rapid pulse;
oliguria;
the formation of stones in the gallbladder and bile ducts.

It should be noted that some patients report an exacerbation of the disease in the cold season. Doctors advise such patients not to overcool.

Prevention

Preventive measures are primary and secondary.

There are many varieties of anemia, some of which do not affect the functioning of the body and the well-being of a person at all. 11% is the number of all anemias, of which 5% are hemolytic characteristics of anemia. Symptoms of hemolytic anemia have their own characteristics, which distinguish this species from other types of disease. Causes are often noted as hereditary and acquired. Treatment is carried out exclusively by a doctor.

Hemolytic anemia is a blood disease in which there is a decrease in the level of red blood cells and hemoglobin in the blood. This is associated with their destruction or hemolysis (short duration of functioning). If normally, red blood cells should function for 120 days, then with hemolytic anemia they are destroyed ahead of time.

The severity of the hemolytic process depends on how quickly the erythrocytes are destroyed. The number of red blood cells and hemoglobin is marked by the fact that the bone marrow simply does not have time to produce new cells.

Thus, with a mild form of hemolytic anemia, the level of red blood cells decreases, but in the peripheral blood, the level of hemoglobin may not be affected. If there is a clear imbalance between the production of red blood cells and their number in the circulating blood, then all the symptoms of the disease appear, in which the functions of the bone marrow are depleted.

Autoimmune hemolytic anemia

The most obscure form of hemolytic anemia is autoimmune. With this form of the disease, the body's antibodies are attached to the membrane of red blood cells, which is why the immune system begins to perceive these cells as foreign. As a result, the immune system attacks red blood cells, destroying them, which leads to a decrease in their number in the blood.

Why develops given form anemia?. However, there are two causes of autoimmune hemolytic anemia:

Complications: hemoblastosis, ulcerative colitis nonspecific, chronic hepatitis aggressive nature, systemic diseases connective tissue, malignant neoplasms, immunodeficiency state, liver cirrhosis, infections.
as an independent disease.

The disease has a progressive nature of the slow type. Clinical manifestations do not depend on the causes of its occurrence. Thus, the first symptoms of autoimmune hemolytic anemia are subfebrile temperature, aching pain in the joints, weakness and abdominal pain. Then the symptomatology intensifies and manifests itself in severe pallor and pastosity of the skin, increasing jaundice, and an increase in the size of the liver and spleen.

In 50% of cases, the disease manifests itself in an acute form, which develops rapidly. The patient may complain, but on examination, the first signs may not be expressed. The patient's complaints are:

Cardiopalmus.
Decreased performance.
Increasing weakness.
Headache.
The temperature rises to 38-39 degrees.
Dizziness.
Lack of air.
Nausea and vomiting that occur without eating food.
Pain in the upper abdomen of a girdle character.

Externally, yellowness of the skin may increase without an increase in the size of the liver and spleen.

The prognosis for autoimmune hemolytic anemia is poor. There are no methods of effective treatment. However, there are ways to achieve a stable remission of the disease - radical splenectomy and hormonal drugs.

Causes of hemolytic anemia

Unfortunately, even knowing the cause of hemolytic anemia, doctors cannot always act on it in order to cure the patient. However, knowing the causes of the disease can help prevent its development.

Hereditary defects that are displayed in the chromosome set responsible for the synthesis and vital activity of red blood cells. This defect is transmitted from parents selectively.
Systemic or autoimmune diseases, which affect the state of the connective tissue and vascular space.
Infectious diseases (malaria).
Blood diseases such as leukemia.
Massive burns or trauma.
Operational intervention.
Viral or bacterial diseases in acute or chronic form.
Contact with industrial poisons or toxic substances.
Rh-conflict pregnancy.
Taking certain medications: antibiotics, chemotherapy drugs, anti-inflammatory drugs, sulfonamides.
Incorrect blood transfusion according to the Rh factor or the group of belonging and its components (plasma, erythrocyte mass, etc.).
Congenital heart defects, main vessels.
Artificial tissue prostheses that come into contact with blood.
Bacterial endocarditis is a disease of the valves and the inner layer of the heart.
Diseases of the vessels of the microcirculatory bed.
Paroxysmal nocturnal hemoglobinuria and cold hemoglobinuria provoke chronic form hemolytic anemia.

Symptoms of hemolytic anemia

It is important for the layman to recognize the presence of hemolytic anemia. This is determined by the following symptoms:

Jaundice syndrome, which manifests itself in a lemon-yellow skin color and itchy sensations. Urine becomes dark and even black, similar to meat slops. In this case, the feces remain unchanged, which distinguishes the disease from jaundice.
anemia syndrome. The skin and mucous membranes become pale. There are symptoms of oxygen starvation: dizziness, palpitations, decreased muscle strength, weakness, shortness of breath.
Syndrome of hyperthermia. A sudden rise to 38 degrees in temperature at the moment when the destruction of red blood cells occurs.
Hepatosplenomegaly syndrome. An increase in the organs that are responsible for the lifespan of red blood cells - the liver and spleen. To a lesser extent, the liver increases, which is marked by heaviness in the right hypochondrium. The spleen increases depending on the degree of hemolysis.

Other symptoms of hemolytic anemia are:

Pain in the bones and abdomen.
Pain in the kidneys.
Loose stool.
Violation prenatal development: malformations, disproportion various parts body.
Chest pain resembling a myocardial infarction.

Signs appear with a life expectancy of erythrocytes for 15 days instead of 120. According to the clinical course, latent (compensated), chronic (with severe anemia) and crisis type of hemolytic anemia are distinguished. Crisis hemolytic anemia is the most severe.

Hemolytic anemia in children

With congenital or hereditary hemolytic anemia, symptoms appear almost from birth. Symptoms in children do not differ from the type of anemia, but careful care and treatment is required. Fortunately, hemolytic anemia occurs in 2 cases per 100,000.

Hemolytic anemia Minkowski-Choffard is the result of a defective gene, as a result of which red blood cells change their shape, becoming more permeable to the sodium ion. The disease is expressed by anemic symptoms and anomalies in the development of the body. The prognosis of life becomes comforting after a radical splenectomy.

Another form of hemolytic anemia is a disease with a lack of G-6-PD activity. Hemolysis occurs after eating legumes or taking certain medications. Symptoms resemble hemolytic anemia, the hallmark of which is the manifestation of hemosiderinuria and hemoglobinuria.

Thalassemia is a common form of genetic hemolytic anemia in which there is excessive accumulation of globin, which leads to premature oxidation and destruction of the red blood cell membrane. The disease manifests itself in anemic syndrome, as well as in physical, psychomotor development. Death large enough due to the constant progression of the disease and the absence of periods of remission.

Treatment of hemolytic anemia

The course of treatment for hemolytic anemia is the most difficult, compared with other types of anemia, due to the inability of doctors to influence the processes of hemolysis. The treatment plan may include:

Reception of cytostatics in autoimmune hemolytic anemia.
Transfusion human immunoglobulin and fresh frozen plasma.
Vitamin B12 and folic acid intake.
Reception of glucocorticoid hormones: Methylprednisolone, Dexamethasone, Cortinef, Prednisolone.
Prevention of complications of an infectious nature and exacerbation of chronic pathology.
Hemotransfusion of open erythrocytes with a decrease in their number to a minimum level.
Splenectomy is the removal of the spleen, which helps in improving prognosis. Not effective for various hereditary types of anemia and Minkowski-Choffard anemia.

Forecast

Which doctors give predictions for hemolytic anemia? It depends on the methods of treatment and their effectiveness in a particular case. Life expectancy can either increase or decrease as the disease progresses.

Hemolytic anemia is a rather rare blood disorder characterized by shortening of the life cycle of red blood cells (erythrocytes). Normally, an erythrocyte lives on average from 3 to 4 months, but in the presence of anemia, this period is reduced to two weeks. Every day certain part red blood cells are replaced by new ones produced by red bone marrow.

If the lifespan of red blood cells is shortened, then replacement cells simply will not have time to mature. It is because of this that their concentration in the peripheral blood is significantly reduced.

Varieties

All existing varieties of hemolytic anemia are divided into two large groups:

hereditary;
acquired.

hereditary forms

non-spherocytic hemolytic anemia. In this case, the cause of destruction is the defective activity of the enzymes responsible for their life cycle;
microspherocytic hemolytic anemia. The reason for the development of such a pathology is the transfer of mutated genes, the main task of which is to synthesize protein molecules that form the wall of red blood cells. In the case of progression of microspherocytic hemolytic anemia, the structure of red blood cells, their activity, as well as resistance to destruction, is significantly reduced;
sickle cell. The reason for the appearance of such a hereditary disease is a gene mutation, the main task of which is to encode the sequence of amino acids in the production of hemoglobin. A characteristic feature of the disease is the deformation of the erythrocyte in the form of a sickle. Affected cells can no longer fully change their shape while passing through the blood vessels, and therefore their increased destruction occurs;
thalassemia. This group of hemolytic anemias occurs due to a violation of the process of hemoglobin production.

Acquired Forms

autoimmune hemolytic anemia. Hemolysis of red blood cells occurs due to the formation of antibodies that accumulate on their membranes. As a result, such red blood cells become marked and macrophages begin to perceive them as foreign agents. In autoimmune acquired hemolytic anemia, the human immune system itself destroys red blood cells;
anemia that occurs with Rhesus conflict and hemolytic disease of newborns. The reason for the progression is the Rh incompatibility of the blood of a woman and her fetus. In the body of the expectant mother, antibodies to the red blood cells of the fetus, which contain the Rh antigen, begin to gradually form. As a result, immune complexes are formed, which lead to the destruction of erythrocyte cells;
traumatic hemolytic anemia. Causes of progression - vessels, the presence of vascular prostheses, changes in the structure of capillaries;
hemolysis of erythrocytes, progressing under the influence of endogenous and exogenous factors;
acute paroxysmal nocturnal hemoglobulinemia.

Etiology

Etiological factors that provoke the progression of autoimmune, sickle cell and other types of hemolytic anemia in children and adults:

various varieties;
autoimmune diseases;
injury varying degrees gravity;
previously transferred pathologies of an infectious nature;
thermal and chemical burns;
surgical interventions;
infectious diseases ();
amyloidosis of vital organs;
prolonged contact with toxic substances;
long-term use of certain groups of synthetic medicines;
transfusion incompatible blood by Rh factor;
Rhesus conflict during childbearing;
bacterial and so on.

Symptoms

For hemolytic anemias (sickle-shaped, autoimmune, non-spherocytic and others), the following symptoms are characteristic:

hyperthermia syndrome. Most often, this symptom manifests itself with the progression of hemolytic anemia in children. Temperature indicators increase to 38 degrees;
syndrome. The skin becomes yellow, but there is no severe itching. characteristic symptom- change in color of urine. It becomes dark and resembles meat slops. Excrement does not change its color;
enlarged liver and spleen syndrome ();
syndrome. The skin and mucous membranes turn pale. Symptoms of hypoxia appear - shortness of breath, weakness, headache, dizziness, and so on.

Additional symptoms of this pathological condition:

pain syndrome at the site of the projection of the kidneys;
pain in the abdomen;
pain in the sternum;
stool change.

Diagnostics

If symptoms appear that indicate the progression of this disease, it is necessary to visit a medical institution for diagnosis and accurate diagnosis. Most informative method detection of hemolytic anemia is. With its help, the doctor will be able to clarify the total number of red blood cells, their quality. A blood test can reveal:

acceleration ;
decrease in concentration;
the presence of deformed erythrocytes;
decrease in the total number of red blood cells;
increase in concentration.

Additional diagnostic methods:

puncture of the red bone marrow.

Treatment

Treatment of hemolytic anemia should be carried out only by a highly qualified specialist. The thing is that this type of anemia is the most difficult to treat, since it is not always possible to eliminate the mechanism for triggering hemolysis.

The pathology treatment plan usually includes the following activities:

the appointment of drugs containing vitamin B12 and folic acid;
hemotransfusion of washed erythrocytes. This method of treatment is resorted to in case of a decrease in the concentration of red blood cells to critical levels;
transfusion of plasma and human immunoglobulin;
to eliminate unpleasant symptoms and normalize the size of the liver and spleen, it is indicated to use glucocorticoid hormones. Dosage Data medicines prescribes only a doctor based on the general condition of the patient, as well as the severity of his illness;
with autoimmune hemolytic anemia, the treatment plan is supplemented with cytostatics;
sometimes doctors resort to surgical methods of treating the disease. The most common is a splenectomy.

Treatment of pathology is carried out only in stationary conditions so that doctors can constantly monitor the general condition of the patient, and prevent the progression of dangerous complications.

Prevention

All preventive actions With this disease, they are divided into primary and secondary. Primary prevention measures are aimed primarily at preventing the progression of hemolytic anemia. Secondary prevention includes measures that will help reduce the manifestation of symptoms of an already progressing pathology.

Primary prevention of autoimmune anemia:

avoid associated infections;
not to be in places with a high content of toxic substances in the air, as well as salts of heavy metals;
timely treatment of infectious diseases.

In case of G-6-PDG deficiency, the following groups of medical drugs should not be used, as they provoke hemolysis:

sulfonamides;
antimalarial drugs;
anti-tuberculosis drugs;
painkillers;
antipyretic;
antibacterial drugs;
drugs from other groups - ascorbic acid, methylene blue, etc.

Secondary prevention of the disease consists in the timely and complete treatment of infectious pathologies that can provoke an exacerbation of hemolytic anemia. To this end, it is recommended to regularly preventive examinations from specialists, as well as to take all the necessary tests.

Is everything correct in the article from a medical point of view?

Answer only if you have proven medical knowledge

Which are characterized by a decrease in the lifespan of red blood cells and their accelerated destruction (hemolysis, erythrocytolysis) either inside the blood vessels or in the bone marrow, liver or spleen.

The life cycle of red blood cells in hemolytic anemia is 15–20 days

Fine average duration the life of erythrocytes is 110–120 days. With hemolytic anemia, the life cycle of red blood cells is shortened several times and is 15-20 days. The processes of destruction of erythrocytes prevail over the processes of their maturation (erythropoiesis), as a result of which the concentration of hemoglobin in the blood decreases, the content of erythrocytes decreases, that is, anemia develops. Others common features characteristic of all types of hemolytic anemia are:

fever with chills;
pain in the abdomen and lower back;
microcirculation disorders;
splenomegaly (enlargement of the spleen);
hemoglobinuria (presence of hemoglobin in the urine);

Hemolytic anemia affects approximately 1% of the population. In the general structure of anemias, hemolytic ones account for 11%.

Causes of hemolytic anemia and risk factors

Hemolytic anemias develop either under the influence of extracellular (external) factors, or as a result of defects in red blood cells (intracellular factors). In most cases, extracellular factors are acquired, while intracellular factors are congenital.

Erythrocyte defects - an intracellular factor in the development of hemolytic anemia

Intracellular factors include abnormalities in erythrocyte membranes, enzymes, or hemoglobin. All of these defects are heritable, with the exception of paroxysmal nocturnal hemoglobinuria. Currently, over 300 diseases associated with point mutations in genes encoding globin synthesis have been described. As a result of mutations, the shape and membrane of erythrocytes change, and their susceptibility to hemolysis increases.

A larger group is represented by extracellular factors. Red blood cells are surrounded by the endothelium (inner lining) of blood vessels and plasma. The presence of infectious agents, toxic substances, and antibodies in the plasma can cause changes in the walls of erythrocytes, leading to their destruction. This mechanism develops, for example, autoimmune hemolytic anemia, hemolytic transfusion reactions.

Defects in the endothelium of blood vessels (microangiopathy) can also damage red blood cells, leading to the development of microangiopathic hemolytic anemia, which is acute in children, in the form of hemolytic uremic syndrome.

Some medications can also cause hemolytic anemia. medicines in particular antimalarials, analgesics, nitrofurans and sulfonamides.

Provoking factors:

vaccination;
autoimmune diseases (nonspecific ulcerative colitis, systemic lupus erythematosus);
some infectious diseases (viral pneumonia, syphilis, toxoplasmosis, infectious mononucleosis);
enzymopathies;
hemoblastoses (multiple myeloma, lymphogranulomatosis, chronic lymphocytic leukemia, acute leukemia);
poisoning with arsenic and its compounds, alcohol, poisonous mushrooms, acetic acid, heavy metals;
heavy physical exercise(long ski crossing, running or walking long distances);
malignant arterial hypertension;
burn disease;
prosthetics of vessels and valves of the heart.

Forms of the disease

All hemolytic anemias are divided into acquired and congenital. Congenital or hereditary forms include:

erythrocyte membranopathies- the result of anomalies in the structure of erythrocyte membranes (acanthocytosis, ovalocytosis, microspherocytosis);
enzymopenia (enzymopenia)- associated with a lack of certain enzymes in the body (pyruvate kinase, glucose-6-phosphate dehydrogenase);
hemoglobinopathies- due to a violation of the structure of the hemoglobin molecule (sickle cell anemia, thalassemia).

The most common in clinical practice hereditary hemolytic anemia is Minkowski-Shoffard disease (microspherocytosis).

Acquired hemolytic anemia, depending on the causes that caused them, are divided into the following types:

acquired membranopathies(spur cell anemia, paroxysmal nocturnal hemoglobinuria);
isoimmune and autoimmune hemolytic anemias- develop as a result of damage to erythrocytes by their own or externally obtained antibodies;
toxic- accelerated destruction of red blood cells occurs due to exposure to bacterial toxins, biological poisons or chemicals;
hemolytic anemia associated with mechanical damage to red blood cells(marching hemoglobinuria, thrombocytopenic purpura).

All types of hemolytic anemia are characterized by:

anemic syndrome;
enlargement of the spleen;
development of jaundice.

At the same time, each separate view disease has its own characteristics.

Hereditary hemolytic anemias

The most common hereditary hemolytic anemia in clinical practice is Minkowski-Choffard disease (microspherocytosis). It is traced through several generations of the family and is inherited in an autosomal dominant manner. A genetic mutation leads to an insufficient content of a certain type of proteins and lipids in the erythrocyte membrane. In turn, this causes changes in the size and shape of erythrocytes, their premature massive destruction in the spleen. Microspherocytic hemolytic anemia can manifest in patients at any age, but most often the first symptoms of hemolytic anemia occur at 10–16 years of age.

Microspherocytosis is the most common hereditary hemolytic anemia.

The disease can proceed with different severity. Some patients have a subclinical course, while others develop severe forms, accompanied by frequent hemolytic crises, which have the following manifestations:

increase in body temperature;
chills;
general weakness;
pain in the lower back and abdomen;
nausea, vomiting.

The main symptom of microspherocytosis is varying degrees expression of jaundice. Because of high content stercobilin (the end product of heme metabolism), feces are intensely stained in dark brown color. In all patients suffering from microspherocytic hemolytic anemia, the spleen is enlarged, and in every second the liver is also enlarged.

Microspherocytosis increases the risk of calculus formation in the gallbladder, i.e., the development of gallstone disease. In this regard, biliary colic often occurs, and when the bile duct is blocked by a stone, obstructive (mechanical) jaundice occurs.

In the clinical picture of microspherocytic hemolytic anemia in children, there are other signs of dysplasia:

bradydactyly or polydactyly;
gothic sky;
malocclusion;
saddle nose deformity;
tower skull.

In elderly patients, due to the destruction of erythrocytes in the capillaries of the lower extremities, trophic ulcers of the feet and legs that are resistant to traditional therapy occur.

Hemolytic anemia associated with a deficiency of certain enzymes usually manifests after taking certain medications or suffering an intercurrent illness. Their characteristic features are:

pale jaundice ( pale color skin with a lemon tint);
heart murmurs;
moderately expressed hepatosplenomegaly;
dark color of urine (due to intravascular breakdown of erythrocytes and excretion of hemosiderin with urine).

In severe cases of the disease, pronounced hemolytic crises occur.

Congenital hemoglobinopathies include thalassemia and sickle cell anemia. The clinical picture of thalassemia is expressed by the following symptoms:

hypochromic anemia;
secondary hemochromatosis (associated with frequent blood transfusions and unreasonable prescription of iron-containing drugs);
hemolytic jaundice;
splenomegaly;
cholelithiasis;
joint damage (arthritis, synovitis).

Sickle cell anemia occurs with recurrent pain crises, moderately severe hemolytic anemia, and increased susceptibility of the patient to infectious diseases. The main symptoms are:

lag of children in physical development (especially boys);
trophic ulcers of the lower extremities;
moderate jaundice;
pain crises;
aplastic and hemolytic crises;
priapism (not related to sexual arousal) spontaneous erection penis, lasting for several hours);
cholelithiasis;
splenomegaly;
avascular necrosis;
osteonecrosis with the development of osteomyelitis.

Acquired hemolytic anemia

Of the acquired hemolytic anemias, autoimmune ones are the most common. Their development leads to the development by the immune system of patients of antibodies directed against their own red blood cells. That is, under the influence of certain factors, the activity of the immune system is disrupted, as a result of which it begins to perceive its own tissues as foreign and destroy them.

At autoimmune anemia hemolytic crises develop suddenly and acutely. Their occurrence may be preceded by precursors in the form of arthralgia and / or subfebrile body temperature. Symptoms of a hemolytic crisis are:

increase in body temperature;
dizziness;
severe weakness;
dyspnea;
heartbeat;
pain in the lower back and epigastrium;
rapid increase in jaundice, not accompanied by itching of the skin;
enlargement of the spleen and liver.

There are forms of autoimmune hemolytic anemia in which patients do not tolerate cold well. With hypothermia, they develop hemoglobinuria, cold urticaria, Raynaud's syndrome (severe spasm of the arterioles of the fingers).

Features of the clinical picture toxic forms hemolytic anemias are:

rapidly progressive general weakness;
high body temperature;
vomit;
severe pain in the lower back and abdomen;
hemoglobinuria.

For 2-3 days from the onset of the disease, the patient begins to increase the level of bilirubin in the blood and develops jaundice, and after 1-2 days hepatorenal insufficiency occurs, manifested by anuria, azotemia, fermentemia, hepatomegaly.

Another form of acquired hemolytic anemia is hemoglobinuria. With this pathology, there is a massive destruction of red blood cells inside the blood vessels and hemoglobin enters the plasma, and then begins to be excreted in the urine. The main symptom of hemoglobinuria is dark red (sometimes black) urine. Other manifestations of pathology can be:

Strong headache ;
a sharp increase in body temperature;
stunning chills;

Hemolysis of erythrocytes in hemolytic disease of the fetus and newborns is associated with the penetration into the bloodstream of the fetus through the placenta of antibodies from the mother's blood, i.e., according to the pathological mechanism, this form of hemolytic anemia belongs to isoimmune diseases.

Normally, the average lifespan of erythrocytes is 110-120 days. With hemolytic anemia, the life cycle of red blood cells is shortened several times and is 15-20 days.

Hemolytic disease of the fetus and newborn can occur in one of the following ways:

intrauterine fetal death;
edematous form ( immune form hydrocele of the fetus);
icteric form;
anemic form.

Common features characteristic of all forms of this disease are:

hepatomegaly;
splenomegaly;
an increase in erythroblasts in the blood;
normochromic anemia.

Diagnostics

Examination of patients with hemolytic anemia is carried out by a hematologist. When questioning the patient, they find out the frequency of formation of hemolytic crises, their severity, and also specifies the presence similar diseases in family history. During the examination of the patient, attention is paid to the color of the sclera, visible mucous membranes and skin, the abdomen is palpated in order to identify a possible enlargement of the liver and spleen. Confirm hepatosplenomegaly allows ultrasound of the abdominal organs.

Changes in the general blood test in hemolytic anemia are characterized by hypo- or normochromic anemia, reticulocytosis, thrombocytopenia, reveal hemoglobinuria, hemosiderinuria, urobilinuria, proteinuria. In the feces there is an increased content of stercobilin.

If necessary, perform a puncture biopsy of the bone marrow, followed by histological analysis(detect hyperplasia of the erythroid germ).

Hemolytic anemia affects approximately 1% of the population. In the general structure of anemias, hemolytic ones account for 11%.

Differential diagnosis of hemolytic anemia is carried out with the following diseases:

hemoblastosis;
hepatolienal syndrome;
portal hypertension;
cirrhosis of the liver;

Treatment of hemolytic anemias

Approaches to the treatment of hemolytic anemia are determined by the form of the disease. But in any case, the primary task is to eliminate the hemolyzing factor.

Treatment regimen for hemolytic crisis:

intravenous infusion of electrolyte and glucose solutions;
transfusion of fresh frozen blood plasma;
vitamin therapy;
prescription of antibiotics and/or corticosteroids (if indicated).

With microspherocytosis, surgical treatment is indicated - removal of the spleen (splenectomy). After surgery, 100% of patients experience stable remission, as the increased hemolysis of erythrocytes stops.

Therapy of autoimmune hemolytic anemia is carried out with glucocorticoid hormones. If it is not effective enough, it may be necessary to prescribe immunosuppressants, antimalarial drugs. Resistance to drug therapy is an indication for splenectomy.

With hemoglobinuria, transfusion of washed erythrocytes, infusion of solutions of plasma substitutes are carried out, antiplatelet agents and anticoagulants are prescribed.

Treatment of toxic forms of hemolytic anemia requires the introduction of antidotes (if any), as well as the use of extracorporeal detoxification methods (forced diuresis, peritoneal dialysis, hemodialysis, hemosorption).

Possible consequences and complications

Hemolytic anemia can lead to the development of the following complications:

heart attacks and rupture of the spleen;
DIC;
hemolytic (anemic) coma.

Forecast

With timely and adequate treatment of hemolytic anemia, the prognosis is generally favorable. At accession of complications it considerably worsens.

Prevention

Prevention of the development of hemolytic anemia includes the following activities:

medical genetic counseling for couples with a family history of indications of cases of hemolytic anemia;
determination of the blood type and Rh factor of the future mother at the stage of pregnancy planning;
strengthening the immune system.

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