IV. Autoimmune hemolytic anemia (Aiga)

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What is Autoimmune Hemolytic Anemia -

Entitled hemolytic anemia unites a group of acquired and hereditary diseases characterized by increased intracellular or intravascular destruction of red blood cells.

Autoimmune hemolytic anemias include forms of the disease associated with the formation of antibodies to self-antigens of erythrocytes.

In the general group of hemolytic anemias, autoimmune hemolytic anemias are more common. Their frequency is 1 case per 75,000-80,000 population.

What provokes / Causes of Autoimmune hemolytic anemia:

Immune hemolytic anemia can occur under the influence of anti-erythrocyte iso- and autoantibodies and, accordingly, are divided into isoimmune and autoimmune.

Isoimmune include hemolytic anemia of newborns, due to incompatibility in the ABO and Rh systems between the mother and fetus, post-transfusion hemolytic anemia.

With autoimmune hemolytic anemia, there is a breakdown of immunological tolerance to unchanged antigens of one's own erythrocytes, sometimes to antigens that have determinants similar to erythrocytes. Antibodies to such antigens are able to interact with unchanged antigens of their own erythrocytes. Incomplete heat agglutinins are the most common type of antibody that can cause the development of autoimmune hemolytic anemia. These antibodies belong to IgG, rarely - to IgM, IgA.

Immune hemolytic anemias are divided into isoimmune and autoimmune. The serological principle of differentiation of autoimmune hemolytic anemias makes it possible to distinguish forms caused by incomplete thermal agglutinins, thermal hemolysins, cold agglutinins, biphasic cold hemolysins (Donat-Landsteiner type) and erythroopsonins. Some authors distinguish a form of hemolytic anemia with antibodies against normoblast antigen. bone marrow.

According to the clinical course, acute and chronic variants are distinguished.

There are symptomatic and idiopathic autoimmune hemolytic anemias. Symptomatic autoimmune anemia occurs against the background of various diseases accompanied by disorders in the immunocompetent system. Most often they are found in chronic lymphocytic leukemia, lymphogranulomatosis, acute leukemia, systemic lupus erythematosus, rheumatoid arthritis, chronic hepatitis and cirrhosis of the liver. In cases where the appearance of autoantibodies cannot be associated with any pathological process, they speak of idiopathic autoimmune hemolytic anemia, which accounts for about 50% of all autoimmune anemias.

The formation of autoantibodies occurs as a result of a violation in the system of immunocompetent cells that perceive the erythrocyte antigen as foreign and begin to produce antibodies to it. After fixation of autoantibodies on erythrocytes, the latter are captured by cells of the reticulohistiocytic system, where they undergo agglutination and decay. Hemolysis of erythrocytes occurs mainly in the spleen, liver, and bone marrow. Autoantibodies to erythrocytes belong to different types.

By sero principle autoimmune hemolytic anemias are divided into several forms:
- anemia with incomplete heat agglutinins
- anemia with thermal hemolysins
- anemia with complete cold agglutinins
- anemia with biphasic hemolysins
- anemia with agglutinins against bone marrow normoblasts

Each of these forms has some features in clinical picture, flow and serological diagnosis. The most common anemia with incomplete thermal agglutinins, accounting for 70 - 80% of all autoimmune hemolytic anemia.

Pathogenesis (what happens?) during Autoimmune hemolytic anemia:

The essence of autoimmune processes is that as a result of the weakening of the T-suppressor system of immunity that controls autoaggression, the B-system of immunity is activated, synthesizing antibodies against unchanged antigens of various organs. T-lymphocytes-killers also take part in the implementation of autoaggression. Antibodies are immunoglobulins (Ig), most often belonging to class G, less often - M and A; they are specific and directed against a particular antigen. IgM include, in particular, cold antibodies and biphasic hemolysins. An erythrocyte carrying antibodies is phagocytosed by macrophages and destroyed in them; possible lysis of erythrocytes with the participation of complement. Antibodies class IgM can cause erythrocyte agglutination directly in the bloodstream, and IgG class antibodies can only destroy erythrocytes in spleen macrophages. In all cases, hemolysis of erythrocytes occurs more intensely, the more antibodies are on their surface. Hemolytic anemia with antibodies to spectrin has been described.

Symptoms of Autoimmune Hemolytic Anemia:

With an acute onset of autoimmune hemolytic anemia, patients develop rapidly increasing weakness, shortness of breath and palpitations, pain in the heart area, sometimes in the lower back, fever and vomiting, intense jaundice. In the chronic course of the process, a relatively satisfactory state of health of patients is noted even with deep anemia, often severe jaundice, in most cases an increase in the spleen, sometimes the liver, alternating periods of exacerbation and remission.

Anemia is normochromic, sometimes hyperchromic, with hemolytic crises usually marked by severe or moderate reticulocytosis. Macrocytosis and microspherocytosis of erythrocytes are found in the peripheral blood, the appearance of normoblasts is possible. ESR is increased in most cases. The content of leukocytes in the chronic form is normal, in the acute form - leukocytosis occurs, sometimes reaching high numbers with a significant shift leukocyte formula to the left. The platelet count is usually normal.
In Fisher-Evens syndrome, autoimmune hemolytic anemia is combined with autoimmune thrombocytopenia. In the bone marrow, erythropoiesis is enhanced, megaloblasts are rarely detected. In most patients, the osmotic resistance of erythrocytes is reduced, which is due to a significant number of microspherocytes in the peripheral blood. The content of bilirubin is increased due to the free fraction, and the content of stercobilin in feces is also increased.

Incomplete heat agglutinins are detected using a direct Coombs test with polyvalent antiglobulin serum. With a positive test using antisera to IgG, IgM, etc., it is specified to which class of immunoglobulins the detected antibodies belong. If there are less than 500 fixed IgG molecules on the surface of red blood cells, the Coombs test is negative. A similar phenomenon is usually observed in patients with a chronic form of autoimmune hemolytic anemia or who have undergone acute hemolysis. Coombs-negative are also cases when antibodies belonging to IgA or IgM are fixed on erythrocytes (in relation to which polyvalent antiglobulin serum is less active).
Approximately 50% of cases of idiopathic autoimmune hemolytic anemia simultaneously with the appearance of immunoglobulins fixed on the surface of erythrocytes, antibodies to their own lymphocytes are detected.

Hemolytic anemia due to thermal hemolysins, is rare. It is characterized by hemoglobinuria with black urine, alternating periods of acute hemolytic crisis and remissions. The hemolytic crisis is accompanied by the development of anemia, reticulocytosis (in some cases, thrombocytosis) and an enlarged spleen. There is an increase in the level of free fraction of bilirubin, hemosiderinuria. When treating donor erythrocytes with papain, it is possible to detect monophasic hemolysins in patients. Some patients have a positive Coombs test.

Hemolytic anemia due to cold agglutinins(cold hemagglutinin disease) chronic course. It develops with a sharp increase in the titer of cold hemagglutinins. There are idiopathic and symptomatic forms of the disease. The leading symptom of the disease is excessive hypersensitivity to cold, which manifests itself in the form of blue and whitening of the fingers and toes, ears, tip of the nose. Disorders of the peripheral circulation lead to the development of Raynaud's syndrome, thrombophlebitis, thrombosis and trophic changes up to acrogangrene, sometimes cold urticaria. The occurrence of vasomotor disorders is associated with the formation of large intravascular conglomerates from agglutinated erythrocytes during cooling, followed by spasm vascular wall. These changes are combined with increased predominantly intracellular hemolysis. In some patients there is an increase in the liver and spleen. Moderately severe normochromic or hyperchromic anemia, reticulocytosis, normal leukocyte and platelet counts, increased ESR, a slight increase in the level of free fraction of bilirubin, a high titer of complete Cold agglutinins (detected by agglutination in a saline medium), sometimes signs of hemoglobinuria are observed. Characteristic is the agglutination of erythrocytes in vitro, which occurs at room temperature and disappears when heated. If it is impossible to perform immunological tests, a provocative test with cooling acquires diagnostic value (in the blood serum obtained from a finger tied with a tourniquet after lowering it into ice water, an increased content of free hemoglobin is determined).

In cold hemagglutinin disease, in contrast to paroxysmal cold hemoglobinuria, the hemolytic crisis and vasomotor disorders occur only from hypothermia of the body and hemoglobinuria, which began in cold conditions, stops when the patient moves to a warm room.

The symptom complex characteristic of cold hemagglutinin disease can occur against the background of various acute infections and some forms of hemoblastoses. With idiopathic forms of the disease, complete recovery is not observed, with symptomatic forms, the prognosis depends mainly on the severity of the underlying process.

Paroxysmal cold hemoglobinuria is one of the rare forms of hemolytic anemia. It affects people of both sexes, more often children.

In patients with paroxysmal cold hemoglobinuria, after being in the cold, general malaise, headache, body aches, and others may appear. discomfort. This is followed by chills, fever, nausea and vomiting. Urine turns black. At the same time, jaundice, enlargement of the spleen and vasomotor disorders are sometimes detected. Against the background of a hemolytic crisis, patients show moderate anemia, reticulocytosis, an increase in the content of the free fraction of bilirubin, hemosiderinuria and proteinuria.

The final diagnosis of paroxysmal cold hemoglobinuria is established on the basis of the detected two-phase hemolysins according to the Donat-Landsteiner method. It is not characterized by autoagglutination of erythrocytes, which is constantly observed in cold hemagglutination disease.

Hemolytic anemia due to erythroopsonins. The existence of autoopsonins to blood cells is generally recognized. With acquired idiopathic hemolytic anemia, cirrhosis of the liver, hypoplastic anemia with a hemolytic component and leukemia, the phenomenon of autoerythrophagocytosis was found.

Acquired idiopathic hemolytic anemia, accompanied by a positive phenomenon of autoerythrophagocytosis, has a chronic course. Periods of remission, sometimes lasting a considerable time, are replaced by a hemolytic crisis, characterized by icterus of visible mucous membranes, darkening of urine, anemia, reticulocytosis and an increase in the indirect fraction of bilirubin, sometimes an increase in the spleen and liver.

In idiopathic and symptomatic hemolytic anemia, the detection of autoerythrophagocytosis in the absence of data indicating the presence of other forms of autoimmune hemolytic anemia gives reason to attribute them to hemolytic anemia caused by erythroopsonins. Diagnostic test of autoerythrophagocytosis is carried out in direct and indirect versions.

Immunohemolytic anemia associated with the use of drugs. Various medical preparations(quinine, dopegyt, sulfonamides, tetracycline, tseporin, etc.), which can cause hemolysis, form complexes with specific heteroantibodies, then settle on erythrocytes and attach complement to themselves, which leads to disruption of the erythrocyte membrane. This mechanism of drug-induced hemolytic anemia is confirmed by the detection of complement on the erythrocytes of patients in the absence of immunoglobulins on them. Anemia is characterized by an acute onset with signs of intravascular hemolysis (hemoglobinuria, reticulocytosis, an increase in the content of the free fraction of bilirubin, increased erythropoiesis). Against the background of a hemolytic crisis, acute renal failure sometimes develops.

Hemolytic anemia, which develops with the appointment of penicillin and methyldopa, proceed somewhat differently. Introduction per day of 15,000 units or more of penicillin can lead to the development of hemolytic anemia, characterized by intracellular hyperhemolysis. Along with the general clinical and laboratory signs of hemolytic syndrome, a positive direct Coombs test is also detected (the detected antibodies are related to IgG). Penicillin, binding to the antigen of the erythrocyte membrane, forms a complex against which antibodies are produced in the body.

With prolonged use of methyldopa, some patients develop a hemolytic syndrome that has the features of an idiopathic form of autoimmune hemolytic anemia. The detected antibodies are identical with thermal agglutinins and belong to IgG.

Hemolytic anemia due to mechanical factors, is associated with the destruction of red blood cells during their passage through altered vessels or through artificial valves. Vascular endothelium changes in vasculitis, malignant arterial hypertension; at the same time, adhesion and aggregation of platelets are activated, as well as the system of blood coagulation and thrombin formation. Widespread blood stasis and thrombosis of small blood vessels (DIC) develop with traumatization of red blood cells, as a result of which they are fragmented; numerous fragments of erythrocytes (schistocytes) are found in a blood smear. RBCs are also destroyed when they pass through artificial valves (more often with multi-valve correction); described hemolytic anemia on the background of senile calcified aortic valve. The diagnosis is based on signs of anemia, an increase in the concentration of free bilirubin in the blood serum, the presence of schistocytes in a peripheral blood smear, and symptoms of the underlying disease that caused mechanical hemolysis.

(Moshkovich's disease, Gasser's syndrome) can complicate the course of autoimmune hemolytic anemia. The disease of an autoimmune nature is characterized by hemolytic anemia, thrombocytopenia, kidney damage. Disseminated lesions of blood vessels and capillaries are noted with the involvement of almost all organs and systems, pronounced changes in the coagulogram, characteristic of DIC.

Diagnosis of autoimmune hemolytic anemia:

Diagnosis of autoimmune hemolytic anemia put on the basis of the presence of clinical and hematological signs of hemolysis and the detection of autoantibodies on the surface of erythrocytes using the Coombs test (positive in almost 60% of autoimmune hemolysis). Differentiate the disease from hereditary microspherocytosis, hemolytic anemia associated with enzyme deficiency.

In the blood - normochromic or moderately hyperchromic anemia of varying severity, reticulocytosis, normoblasts. In some cases, microspherocytes are found in blood smears. The number of leukocytes may increase during a hemolytic crisis. The platelet count is usually within the normal range, but thrombocytopenia may occur. ESR is significantly increased. In the bone marrow, there is marked hyperplasia of the erythroid germ. The content of bilirubin in the blood, as a rule, is increased due to indirect.

Treatment for Autoimmune Hemolytic Anemia:

In acute forms of acquired autoimmune hemolytic anemia, prednisolone is prescribed in a daily dose of 60-80 mg. With inefficiency, it can be increased to 150 mg or more. The daily dose of the drug is divided into 3 parts in a ratio of 3:2:1. As the hemolytic crisis subsides, the dose of prednisolone is gradually reduced (2.5-5 mg per day) to half the original. A further reduction in the dose of the drug in order to avoid recurrence of the hemolytic crisis is carried out at 2.5 mg for 4-5 days, then in smaller doses and at longer intervals until the drug is completely discontinued. In chronic autoimmune hemolytic anemia, it is enough to prescribe 20-25 mg of prednisolone, and as it improves general condition the patient and indicators of erythropoiesis should be transferred to a maintenance dose (5-10 mg). With cold hemagglutinin disease, similar therapy with prednisolone is indicated.

Splenectomy for autoimmune hemolytic anemia associated with thermal agglutinins and autoerythroopsonins can only be recommended for patients in whom corticosteroid therapy is accompanied by short remissions (up to 6-7 months) or there is resistance to it. In patients with hemolytic anemia caused by hemolysins, splenectomy does not prevent hemolytic crises. However, they are observed less frequently than before surgery, and are more easily stopped with the help of corticosteroid hormones.

With refractory autoimmune hemolytic anemia, immunosuppressants (6-mercaptopurine, imuran, chlorbutine, methotrexate, cyclophosphamide, etc.) can be used in combination with prednisolone.

In the stage of a deep hemolytic crisis, transfusions of erythrocyte mass, selected using an indirect Coombs test, are used; to reduce the pronounced endogenous intoxication prescribe hemodez, polydez and other detoxification agents.

Treatment of hemolytic-uremic syndrome, which can complicate the course of autoimmune hemolytic anemia, includes corticosteroid hormones, fresh frozen plasma, plasmapheresis, hemodialysis, transfusion of washed or cryopreserved red blood cells. Despite the use of a complex of modern therapeutic agents, the prognosis is often unfavorable.

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Other diseases from the group Diseases of the blood, hematopoietic organs and individual disorders involving the immune mechanism:

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 does this form of anemia develop? However, there are two causes of autoimmune hemolytic anemia:

1. Complications: hemoblastosis, nonspecific ulcerative colitis, aggressive chronic hepatitis, systemic connective tissue diseases, malignant neoplasms, immunodeficiency, liver cirrhosis, infections.
2. 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 pain in the abdomen. 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. Missing Methods 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 that affect the condition 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:

1. 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.
2. anemia syndrome. The skin and mucous membranes become pale. Symptoms appear oxygen starvation: dizziness, palpitations, decreased muscle strength, weakness, shortness of breath.
3. Syndrome of hyperthermia. A sudden rise to 38 degrees in temperature at the moment when the destruction of red blood cells occurs.
4. 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 of intrauterine development: malformations, disproportion of various parts of the body.
• Pain in the chest, 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. The lethal outcome is quite large 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:

1. Reception of cytostatics in autoimmune hemolytic anemia.
2. Transfusion human immunoglobulin and fresh frozen plasma.
3. Vitamin B12 and folic acid intake.
4. Reception of glucocorticoid hormones: Methylprednisolone, Dexamethasone, Cortinef, Prednisolone.
5. Prevention of complications of an infectious nature and exacerbation of chronic pathology.
6. Hemotransfusion of open erythrocytes with a decrease in their number to a minimum level.
7. 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.

Various pathological processes that occur in the immune system can inevitably lead to serious disorders. An autoimmune disorder is characterized by the fact that the body begins to consider its own cells as "enemy" and begins to fight them. Autoimmune hemolytic anemia belongs to a group of rare diseases in which there is a process of formation of antibodies to one's own red blood cells. The consequences of this phenomenon are quite serious, since violations circulatory system affect the functioning of the whole organism.

All autoimmune pathological disorders appear as a result of malfunctioning immune system. Also, a hereditary factor that predisposes to the appearance of immune diseases is not excluded. In newborns, incompatibility with the mother's Rh is diagnosed. Another reason for the appearance of this disorder may be damage to the DNA chain or due to gene mutation.

Acquired autoimmune hemolytic anemias are classified according to the factor that provoked the progression of the disease. In the case when it is not possible to associate the formation of a provoking factor due to which the rejection of erythrocytes appears, it is customary to diagnose idiopathic anemia. Unspecified anemia accounts for nearly half of all cases.

Symptoms of autoimmune anemia

The manifestations of the disease directly depend on the form, of which there are only two - acute and chronic. The acute form is characterized by the following manifestations:

1. The appearance of rapidly progressive pain.
2. Shortness of breath even with slight exertion.
3. Painful discomfort in the region of the heart.
4. There is an increase in body temperature, vomiting.
5. The appearance of yellowness skin.

The chronic form is outwardly manifested by the relatively satisfied state of the patient, even if the disease has long been neglected. Often there is a yellowness of the skin, with ultrasound, an enlarged spleen is diagnosed. Periods of exacerbation alternate with a calm course of the disease. Code according to the reference book microbial 10 autoimmune hemolytic anemia stands for D59.

With cold autoimmune anemic disease, poor tolerance may occur low temperatures. In the cold, patients show characteristic signs of the disorder: urticaria and Raynaud's syndrome. Raynaud's syndrome is characterized by numbness and pain in the limbs of the hands, the appearance of a bluish tinge of the skin, the tip of the nose, tongue and ears also suffer. With infectious diseases, exacerbation of the anemic syndrome is possible.

Autoimmune hemolytic anemia in children

In young children, a fairly rare form of autoimmune hemolytic anemia is often diagnosed - anemia with biphasic hemolysin. It also has a second name - cold anemia. Characteristic features disorders are the following manifestations: chills, fever, abdominal pain, nausea and bouts of vomiting, with hypothermia, a black tint of urine appears. During an exacerbation, ultrasound reveals an increase in the size of the spleen, an icteric shade of the skin appears.

Diagnostics

Clinical and hematological signs give grounds for the diagnosis of autoimmune hemolytic anemia. Also, for diagnosis, a Coombs test is performed, which reliably displays the patient's condition. The presence of microspherocytes, normoblasts, normochromic anemia is determined by the blood picture. The level of ESR is often significantly elevated, and the level of bilirubin in the blood is also increased.

Treatment of autoimmune hemolytic anemia

For the treatment of autoimmune anemia, it is necessary to consult a hematologist. Autoimmune anemia often occurs with pronounced clinical manifestations, and therefore the specialist prescribes, first of all, drugs with a potent effect. These drugs are designed to suppress the immune system.

All therapeutic measures are carried out for almost three months. A negative Coombs test is considered a productive result. Only after this, treatment therapy is stopped. In some cases, autoimmune anemic pathology is not treatable. Then only surgery is prescribed - removal of the spleen.

In the acute course of the disease, a blood transfusion may be required. The administration of prednisolone is also prescribed for cold anemia or chronic. The disease is quite difficult to treat. Despite all modern medical therapies, the prognosis for autoimmune anemia is often poor.

Prevention

It is not possible to prevent or foresee the occurrence of such a disease, and therefore there are no practical preventive methods. It should be added that the most effective and effective way warning against any disease healthy lifestyle life.

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Autoimmune hemolytic anemia is an autoaggressive disease in which red blood cells are destroyed due to uncontrolled production of antibodies against one's own red blood cells. The disease most often affects women. This type of anemia is diagnosed not on the basis of a blood test. The disease is difficult to treat with the use of immunosuppressants, and often the spleen has to be removed.

Reasons for the development of the disease

It has not been fully established what causes the development of hemolytic anemia. The main provoking factor is the production of antibodies to one's own erythrocytes, which are perceived by the body as foreign antigens. Anemia may occur as a complication of the following diseases:

• chronic aggressive hepatitis;
• hemoblastosis;
• malignant neoplasms;
• nonspecific ulcerative colitis.

In addition, autoimmune anemia can develop due to the use of beta-lactam antibiotics (Ceftriaxone, Augmentin, Amoxiclav, Penicillin). The appearance of anemia is observed during exacerbation of mycoplasma and cytomegalovirus infection.

Varieties and symptoms of the disease

There are the following types of hemolytic anemia:

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• symptomatic, developing against the background of various diseases;
• idiopathic, provoked by reasons that cannot be established.

All manifestations of anemia do not depend on the etiological factor. Often the disease is characterized by a slowly progressive course. The first signs of autoimmune hemolytic anemia include:

• general weakness;
• abdominal pain;
• elevated body temperature;
• aching pain in the joints.

When examining the patient, there is a pronounced pallor of the skin, increasing jaundice, an enlarged liver and spleen.

Often, autoimmune anemia causes symptoms to come on suddenly. The patient begins to complain about the state of health, but during the examination the doctor does not reveal any changes. The main complaints include:

• increased weakness;
• feeling short of breath;
• cardiopalmus;
• decrease in working capacity;
• nausea and vomiting;
• dizziness;
• headache;
• temperature increase up to 39°C;
• girdle aching pain in the upper abdomen.

Diagnostics

Diagnosis of the disease includes an assessment of the patient's complaints, a study of the history of the disease and laboratory tests. The examination is carried out by a hematologist, rheumatologist, infectious disease specialist, allergist-immunologist and other specialists. During the examination, pallor of the skin and mucous membranes is detected, the enlarged liver and spleen are well palpated.

to the most important laboratory research include:

• General blood analysis. Allows you to detect a change in ratio various kinds leukocytes in accordance with the disease that caused autoimmune anemia. Idiopathic anemia is characterized by an increase in the number of monocytes and lymphocytes. With hypochromic anemia, the concentration of hemoglobin in the erythrocyte membrane is disturbed.
• Urinalysis, which reveals hemoglobinuria, urobilinemia, proteinuria.
• Biochemical analysis blood. An increase in total protein indicates inflammatory process; an increase in the amount of indirect bilirubin occurs as a result of the destruction of red blood cells; increase serum iron indicates a high rate of destruction of red blood cells.
• Coombs test. Such an analysis is performed in order to detect antibodies in plasma that are sensitive to erythrocytes. If the reaction is positive, then the diagnosis is confirmed.

Treatment Methods

If autoimmune hemolytic anemia is diagnosed, then treatment should be comprehensive. With a symptomatic form of anemia, they get rid of the disease that caused its development. If anemia is idiopathic, then corticosteroids (glucocorticoids), for example, Prednisolone, are used. Treatment of the disease is carried out by transfusion of washed red blood cells or whole blood.

Blood detoxification is also carried out to remove the breakdown products of red blood cells. Hemolytic autoimmune anemia can be cured with plasmapheresis, which reduces the amount of circulating antibodies in the blood. When prescribing direct and indirect anticoagulants, the likelihood of developing DIC, which accompanies acute blood loss, is prevented.

- pathology of erythrocytes, the hallmark of which is the accelerated destruction of red blood cells with the release of an increased amount of indirect bilirubin. For this group of diseases, a combination of anemic syndrome, jaundice and an increase in the size of the spleen is typical. During the diagnostic process, general analysis blood, bilirubin level, stool and urine analysis, ultrasound of organs abdominal cavity; a bone marrow biopsy, immunological studies are performed. As methods of treatment, drug, blood transfusion therapy is used; with hypersplenism, splenectomy is indicated.

ICD-10

D59 D58

General information

Hemolytic anemia (HA) - anemia caused by a violation life cycle erythrocytes, namely, the predominance of the processes of their destruction (erythrocytolysis) over the formation and maturation (erythropoiesis). This group of anemias is very extensive. Their prevalence is not the same in different geographical latitudes and age cohorts; on average, pathology occurs in 1% of the population. Among other types of anemia, hemolytic ones account for 11%. Pathology is characterized by a shortening of the life cycle of erythrocytes and their decay (hemolysis) ahead of time (after 14-21 days instead of 100-120 days normally). In this case, the destruction of red blood cells can occur directly in the vascular bed (intravascular hemolysis) or in the spleen, liver, bone marrow (extravascular hemolysis).

The reasons

The etiopathogenetic basis of hereditary hemolytic syndromes is genetic defects in erythrocyte membranes, their enzyme systems, or hemoglobin structure. These prerequisites determine the morphofunctional inferiority of erythrocytes and their increased destruction. Hemolysis of erythrocytes in acquired anemia occurs under the influence of internal factors or factors environment, among which:

• Autoimmune processes. The formation of antibodies that agglutinate erythrocytes is possible with hemoblastoses (acute leukemia, chronic lymphocytic leukemia, lymphogranulomatosis), autoimmune pathology (SLE, nonspecific ulcerative colitis), infectious diseases (infectious mononucleosis, toxoplasmosis, syphilis, viral pneumonia). The development of immune hemolytic anemia can be promoted by post-transfusion reactions, preventive vaccination, hemolytic disease of the fetus.
• Toxic effect on erythrocytes. In some cases, acute intravascular hemolysis is preceded by poisoning with arsenic compounds, heavy metals, acetic acid, mushroom poisons, alcohol, etc. Destruction of blood cells can be caused by taking certain medicines(antimalarial drugs, sulfonamides, nitrofuran derivatives, analgesics).
• Mechanical damage to erythrocytes. Hemolysis of erythrocytes can be observed in severe physical activity(long walking, running, skiing), with DIC, malaria, malignant hypertension, prosthetic heart valves and blood vessels, hyperbaric oxygen therapy, sepsis, extensive burns. In these cases, under the influence of certain factors, traumatization and rupture of the membranes of initially full-fledged erythrocytes occur.

Pathogenesis

The central link in the pathogenesis of GA is the increased destruction of erythrocytes in the organs of the reticuloendothelial system (spleen, liver, bone marrow, lymph nodes) or directly in the vascular bed. With the autoimmune mechanism of anemia, the formation of anti-erythrocyte antibodies (heat, cold) occurs, which cause enzymatic lysis of the erythrocyte membrane. Toxic substances, being the strongest oxidizing agents, destroy the erythrocyte due to the development of metabolic, functional and morphological changes in the membrane and stroma of red blood cells. Mechanical factors have a direct effect on the cell membrane. Under the influence of these mechanisms, potassium and phosphorus ions leave the erythrocytes, and sodium ions enter inside. The cell swells, with a critical increase in its volume, hemolysis occurs. The breakdown of erythrocytes is accompanied by the development of anemic and icteric syndromes (the so-called "pale jaundice"). Perhaps intense staining of feces and urine, enlargement of the spleen and liver.

Classification

In hematology, hemolytic anemias are divided into two large groups: congenital (hereditary) and acquired. Hereditary GA includes the following forms:

• erythrocyte membranopathies(- Minkowski-Choffard disease, ovalocytosis, acanthocytosis) - anemia, due to structural abnormalities of erythrocyte membranes
• fermentopenia(enzymopenia) - anemia caused by a deficiency of certain enzymes (glucose-6-phosphate dehydrogenase, pyruvate kinase, etc.)
• hemoglobinopathies- anemia associated with qualitative disturbances in the structure of hemoglobin or a change in the ratio of its normal forms (thalassemia, sickle cell anemia).

Acquired GAs are divided into:

• acquired membranopathies(paroxysmal nocturnal hemoglobinuria - Marchiafava-Mikeli b-b, spur cell anemia)
• immune (auto- and isoimmune)- due to exposure to antibodies
• toxic- anemia due to exposure chemical substances, biological poisons, bacterial toxins
• mechanical- anemia caused by mechanical damage to the structure of red blood cells (thrombocytopenic purpura, marching hemoglobinuria)

Symptoms

Hereditary membranopathies, fermentopenias and hemoglobinopathies

The most common form of this group of anemias is microspherocytosis, or Minkowski-Choffard disease. Inherited in an autosomal dominant manner; usually seen in several members of the family. The defectiveness of erythrocytes is due to a deficiency in the membrane of actomyosin-like protein and lipids, which leads to a change in the shape and diameter of erythrocytes, their massive and premature hemolysis in the spleen. Manifestation of microspherocytic GA is possible at any age (in infancy, adolescence, old age), but usually manifestations occur in older children and adolescents. The severity of the disease varies from sub clinical course before severe forms characterized by frequently recurring hemolytic crises. At the time of the crisis, body temperature rises, dizziness, weakness; abdominal pain and vomiting occur.

The main symptom of microspherocytic hemolytic anemia is jaundice. varying degrees intensity. Due to the high content of stercobilin, the feces become intensely colored in a dark brown color. Patients with Minkowski-Choffard disease have a tendency to form stones in gallbladder therefore, signs of exacerbation of calculous cholecystitis often develop, attacks of biliary colic occur, and when the choledochus is blocked by a calculus, obstructive jaundice occurs. With microspherocytosis, the spleen is enlarged in all cases, and in half of the patients, the liver is also enlarged. In addition to hereditary microspherocytic anemia, other congenital dysplasias often occur in children: tower skull, strabismus, saddle nose deformity, malocclusion, gothic palate, polydactyly or bradydactyly, etc. Middle-aged and elderly patients suffer from trophic leg ulcers that occur as a result of hemolysis of red blood cells in the capillaries of the extremities and are difficult to treat.

Enzymopenic anemias are associated with a lack of certain erythrocyte enzymes (more often - G-6-PD, glutathione-dependent enzymes, pyruvate kinase, etc.). Hemolytic anemia may first manifest itself after an intercurrent illness or medication (salicylates, sulfonamides, nitrofurans). Usually the disease has a smooth course; typical "pale jaundice", moderate hepatosplenomegaly, heart murmurs. In severe cases, a pronounced picture of a hemolytic crisis develops (weakness, vomiting, shortness of breath, palpitations, collaptoid state). In connection with intravascular hemolysis of erythrocytes and the release of hemosiderin in the urine, the latter acquires a dark (sometimes black) color. Peculiarities of the clinical course of hemoglobinopathies - thalassemia and sickle cell anemia are the subject of independent reviews.

Acquired hemolytic anemia

Among the various acquired variants, autoimmune anemias are more common than others. For them, the common starting factor is the formation of antibodies to the antigens of their own erythrocytes. Hemolysis of erythrocytes can be both intravascular and intracellular. Hemolytic crisis in autoimmune anemia develops acutely and suddenly. It proceeds with fever, severe weakness, dizziness, palpitations, shortness of breath, pain in the epigastrium and lower back. Sometimes acute manifestations preceded by precursors in the form of low-grade fever and arthralgia. During the crisis, jaundice is rapidly increasing, not accompanied by skin itching, the liver and spleen are enlarged. In some forms of autoimmune anemia, patients do not tolerate cold well; at low temperatures, they may develop Raynaud's syndrome, urticaria, hemoglobinuria. Due to circulatory failure in small vessels, complications are possible in the form of gangrene of the toes and hands.

Toxic anemia occurs with progressive weakness, pain in the right hypochondrium and lumbar region, vomiting, hemoglobinuria, high body temperature. From 2-3 days jaundice and bilirubinemia join; on the 3-5th day, hepatic and renal failure occurs, the signs of which are hepatomegaly, fermentemia, azotemia, anuria. Separate types of acquired hemolytic anemia are discussed in the relevant articles: "Hemoglobinuria" and "Thrombocytopenic purpura", "Hemolytic disease of the fetus".

Complications

Each type of HA has its own specific complications: for example, cholelithiasis - with microspherocytosis, liver failure - with toxic forms, etc. Common complications include hemolytic crises, which can be triggered by infections, stress, childbirth in women. In acute massive hemolysis, the development of a hemolytic coma is possible, characterized by collapse, confusion, oliguria, and increased jaundice. The patient's life is threatened by DIC, spleen infarction, or spontaneous organ rupture. emergency medical care require acute cardiovascular and renal failure.

Diagnostics

Determining the form of GA based on an analysis of the causes, symptoms and objective data is within the competence of a hematologist. During the initial conversation, the family history, frequency and severity of the course of hemolytic crises are clarified. During the examination, the color of the skin, sclera and visible mucous membranes is evaluated, the abdomen is palpated to assess the size of the liver and spleen. Spleno- and hepatomegaly is confirmed by ultrasound of the liver and spleen. Laboratory diagnostic complex includes:

• Blood test. Changes in the hemogram are characterized by normo- or hypochromic anemia, leukopenia, thrombocytopenia, reticulocytosis, and accelerated ESR. In biochemical blood samples, hyperbilirubinemia is determined (an increase in the fraction of indirect bilirubin), an increase in the activity of lactate dehydrogenase. In autoimmune anemia, a positive Coombs test is of great diagnostic value.
• Urine and stool tests. Urinalysis reveals proteinuria, urobilinuria, hemosiderinuria, hemoglobinuria. The content of stercobilin was increased in the coprogram.
• Myelogram. For cytological confirmation, a sternal puncture is performed. Examination of bone marrow punctate reveals hyperplasia of the erythroid germ.

In the process differential diagnosis hepatitis, cirrhosis of the liver, portal hypertension, hepatolienal syndrome, porphyria, hemoblastoses are excluded. The patient is consulted by a gastroenterologist, clinical pharmacologist, infectious disease specialist and other specialists.

Treatment

Different forms of GA have their own characteristics and approaches to treatment. With all variants of acquired hemolytic anemia, care must be taken to eliminate the influence of hemolytic factors. During hemolytic crises, patients need infusions of solutions, blood plasma; vitamin therapy, if necessary - hormone and antibiotic therapy. With microspherocytosis, the only effective method leading to 100% cessation of hemolysis is splenectomy.

In autoimmune anemia, therapy with glucocorticoid hormones (prednisolone) is indicated, which reduces or stops hemolysis. In some cases, the desired effect is achieved by the appointment of immunosuppressants (azathioprine, 6-mercaptopurine, chlorambucil), antimalarial drugs (chloroquine). When resistant to drug therapy forms of autoimmune anemia, splenectomy is performed. Treatment of hemoglobinuria involves the transfusion of washed red blood cells, plasma substitutes, the appointment of anticoagulants and antiplatelet agents. The development of toxic hemolytic anemia dictates the need for intensive care: detoxification, forced diuresis, hemodialysis, according to indications - the introduction of antidotes.

Forecast and prevention

The course and outcome depend on the type of anemia, the severity of the course of crises, the completeness of pathogenetic therapy. With many acquired variants, the elimination of the causes and the full treatment leads to a complete recovery. Congenital anemia cannot be cured, but long-term remission is possible. With the development kidney failure and other fatal complications, the prognosis is unfavorable. The development of GA can be prevented by the prevention of acute infectious diseases, intoxication, poisoning. Uncontrolled independent use is prohibited medicines. Careful preparation of patients for blood transfusions, vaccination with the entire complex of necessary examinations is necessary.