Symptoms and treatment of hemolytic anemia

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Symptoms and treatment of hemolytic anemia

Thalassemia in a child

Thalassemia is anemia, which is based on a congenital defect in the transport RNA or gene-regulator. Also, the rate of production of one of the globin chains, alpha, beta or gamma, is disturbed. All this, in combination, leads to the development of thalassemia. More often than others, there is a violation in the production of beta-chains, therefore the exposed diagnosis sounds predominantly like beta-thalassemia. The concentration of normal hemoglobin decreases, and the concentration of abnormal hemoglobin increases (HbF and HbA 2).

Erythrocytes, which are transporters of the modified anomalous hemoglobin, have very thin walls, they are tender and quickly decay. Often they are unable to penetrate the capillaries, since their wall is too thin. As a result, a person develops thalassemia symptoms.

There are two variants of the disease:

  • Homozygous or Cooley disease that develops in childhood.

  • Heterozygous form or small thalassemia. The disease is most often detected by chance, during a blood test. Diagnosis of the disorder in adulthood. Small thalassemia may not manifest itself at all, that’s why a person sometimes does not even suspect about the existing disorders in his body. The course of the pathology is easy. Large thalassemia has a severe course, it develops in children of the first year of life, gives multiple complications.

    The following symptoms will indicate Cooley’s illness:

    • Pale skin, its yellow color, which can be very intense.

    • Weakness, fatigue and lethargy.

    • An increase in the size of the spleen, which is especially noticeable in childhood. Also in size, the liver can grow.

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    • The level of erythrocytes in the blood can remain normal, and the hemoglobin level decreases.

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    • The child will be somewhat behind in development, such children later begin to crawl, they have an impaired appetite, the child does not walk well.

    Sometimes Cooley’s disease can be confused with hepatitis. To expose a differential diagnosis, you need to study the family history, as well as pay attention to a blood indicator, such as bilirubin. With Cooley’s disease, the level of free bilirubin, as well as the level of bilirubin fractions of HbF and HbA 2, will be increased. In addition, the blood increases the concentration of reticulocytes, and urobilin appears in the urine.

    Therapy is reduced to the following steps:

    • Transfusion of erythrocyte mass, provided the level of hemoglobin is significantly reduced.

    • Passing the course of desferal therapy, which allows you to prevent hemosiderosis from developing.

    • Removal of the spleen.

    • Taking folic acid.

    • Bone marrow transplantation.

    Ferrotherapy is not prescribed for thalassemia.

    Minkowski-Schoffar’s disease

    Minkowski-Schoffar’s disease (microspherocytic membranopathy) is transmitted by an autosomal dominant type. In this case, the patient has a congenital defect of erythrocyte membranes, under which they acquire a spherical shape. While normal healthy red blood cells have a disc shape. This allows them to penetrate through the narrowest passages of the capillary network. Erythrocytes, having a spherical shape, can not squeeze through small vessels, but when they try to do this, they are destroyed. Moreover, spherical erythrocytes let in more sodium ions than necessary. As a result, red blood cells spend the maximum amount of energy, which also shortens their life span.

    Manifestation pathology can at any age, but the younger the child, the heavier will be its course. Especially dangerous is Minkowski-Schoffar’s disease in the period of newborns.

    Hemolytic syndrome is the main symptom of Minkowski-Schoffar disease. It can develop for no apparent reason, either against the background of increased loads, after the injuries received, or after the expressed supercooling of the organism. Pathology enters the phase of exacerbation, and then gives way to a period of calm. In addition, the disease leads to such anomalies of development, as: a tower skull, a hare lip, defects of internal organs, heart defects.

    Other symptoms of hereditary microspherocytosis:

    • Severe yellowness of the skin. If the patient develops acute hemolytic anemia, then it will be simply impossible not to notice a discoloration of the skin.

    • Unexplained weakness.

    • A high body temperature that reaches peak values ​​during the acute phase.

    • Pain in muscles, pain in right upper quadrant, in abdomen.

    • Tachycardia, a drop in blood pressure, noises in the heart.

    • Increase in spleen in size.

    • Darkening of urine and feces. If at the first examination of the patient the doctor can confuse anemia with viral hepatitis, then the results of laboratory tests this diagnosis is refuted.

      The conducted general blood test will detect a decrease in the level of hemoglobin and red blood cells in the blood. And most of the red blood cells will have the shape of a sphere, and their dimensions decrease.

      In addition, the level of reticulocytes rises, autohemolysis increases, blood bilirubin in the blood becomes larger, and the osmotic resistance of red blood cells decreases. In the analysis of stool, the level of sterocilin will be exceeded, and in the analysis of urine – the level of urobilin.

      If the disease is severe, the patient is transfused with erythrocyte mass. B vitamins and iron preparations with this type of anemia are not prescribed, since their effectiveness will be zero. To improve the patient’s condition, a spleen removal is required, but this operation does not allow to completely solve the problem.

      Other hereditary causes

      Of course, in addition to the two described diseases, there are other hereditary hemolytic anemia. They are rare.

      These diseases include:

      • Serpovidokletochnaya anemia. This disease is similar to thalassemia, but with a crisis of sickle-cell anemia, massive destruction of red blood cells occurs. Hemolysis will also be quite intense. In childhood, this type of anemia can be triggered by infectious diseases. Anemia is associated with a risk of complications such as: edema and lung infarction, paralysis, liver cirrhosis, cardiomegaly. In adulthood crises are observed less often, but the likelihood of severe consequences persists.

      • Ovalocytosis. This hereditary disease is similar to microspherocytosis in terms of the development of symptoms of anemia. However, erythrocytes in ovalocytosis have an oval shape. Deficiency of activity G-6-FDG is a hereditary disease that is based on a disturbance in the production of red blood cells. The course of pathology can be very diverse. Sometimes the symptoms of anemia are barely noticeable, and sometimes the disease provokes severe complications. It develops both in infants and adults. The smaller the child, the more severe will be the disturbances from the nervous system. Crises most often develop against the background of taking medications, during pregnancy, after or during infectious diseases, when ingested allergens. If you do not diagnose such a crisis in time, then the development of DIC-syndrome or acute renal failure is possible, which can result in death. Video: lecture on hemolytic anemia:

        Autoimmune hemolytic anemia

        AIGA or autoimmune hemolytic anemia is pathology, which develops against the background of the production of antibodies to erythrocyte antigens. For some reason, the immune system perceives its own red blood cells for malicious agents and begins to attack them.

        There are 2 types of AIGA:

        • Symptomatic anemia that manifests due to other diseases, for example, against hepatitis, systemic lupus erythematosus, rheumatoid arthritis, Idiopathic anemia, which can be triggered by a variety of causes, but it is more often than not impossible to establish which ones. This can be the transferred injuries, childbirth, infection, taking medicines, etc. Anemia develops due to the fact that the erythrocytes are deposited by haptens, but which one caused such a reaction, it is not possible to find out. Often, AIGA occurs after abortion, after food poisoning, or after taking medications that the doctor did not prescribe. In this regard, self-medication is very dangerous.

          Autoimmune hemolytic anemia develops according to the following scheme:

          • The antigenic structure of red blood cells changes due to the impact of pathological factors on it. It can be allergens, bacteria, viruses, medicinal substances.

          • On the changed erythrocytes, the body begins to direct its own antibodies, with the aim of destroying them. From damaged red blood cells, hemoglobin is released, anemia develops. Antibodies that can lead to an immunological reaction:

            • Incomplete thermal agglutinins. They attach to the surface of red blood cells and begin to actively “work”. At the same time in the blood Affected erythrocytes do not perish, but are sent to “die” in the spleen. Most often such agglutinins are immunoglobulins of class lgG. They show maximum activity at a temperature of 37 degrees.

            • Cold incomplete agglutinins that attack erythrocytes at a temperature of 32 degrees. They destroy the red blood cells when they are in the capillaries. It is in these vessels that the body temperature is lowered to the desired values. Suffer fingers and toes, nose, ears. The class M-IgM belongs to the agglutinin.

            • Two-phase hemolysins that can meet their antigen during cooling of body temperature. They attach to the red blood cells and wait until the body temperature rises to 37 degrees. During this period they begin to show their aggressive qualities, which lead to hemolysis. To such hemolysins belongs class G – IgG.

            Symptoms of autoimmune hemolytic anemia may differ.

            This largely depends on what factors triggered its development:

            • Whether anemia was manifested against the background of a crisis, or against a background of health.

            • Heat or cold is necessary for the aggressor to develop a pathological reaction.

            • Where exactly is the process of the death of red blood cells.

            • Does the person have pathological changes in internal organs.

            • Is there any chronic disease. The main symptoms that will indicate hemolytic anemia are: a hemolytic crisis with yellowing of the skin, an increase in body temperature, an increase in the spleen of. The hemoglobin and erythrocytes in the blood also drop in the patient, while in hemoglobin urine, in contrast, begins to rise.

              Indirect signs of hemolytic autoimmune anemia are: increased weakness and fatigue, enlargement of the liver in size.

              During the diagnosis, the doctor will send the patient to donate blood for biochemical and general analysis, as well as for a general urinalysis. A Kumbas test is mandatory, which provides information on the type of antibodies that attack red blood cells.

              If the patient develops an acute hemolytic anemia of an autoimmune nature, he is placed in a hospital. Treatment requires the administration of corticosteroids and immunosuppressants. If this does not help, then an operation is required to remove the spleen.

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