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Congenital hemolytic anemia

The most studied of these forms is microspherocytic hemolytic anemia - Minkowski-Chauffard disease, which is inherited in an autosomal dominant manner. In the erythrocyte membrane, the content of actomyosin-like protein, phospholipids and cholesterol is reduced, which leads to a decrease in the total surface of erythrocytes and the formation of microcytes. Microcytes have a diameter of up to 6 microns, a thickness of 2.5-3 microns. These small cells are intensely stained on the smear, without central enlightenment. Their osmotic resistance is sharply reduced, as well as their mechanical resistance. The course of the disease is diverse - from light to severe forms, with frequent hemolytic crises. Complaints of patients may be missing. So, according to Chauffard, These patients are more yellow than sick. Patients have yellowness of the skin, sclera and mucous membranes. At the same time, urine is saturated, feces sharply colored. All patients had an enlarged spleen, half of the patients had an enlarged liver. In some patients, congenital anomalies can occur: tower skull, gothic palate, strabismus, malformations of the heart and blood vessels. There are bilateral ulcers on the skin of the legs.

When hemolytic crisis occurs, intense pain in the right and left hypochondria, chills, temperature rise up to 39-40 C, vomiting, increased jaundice and anemia, an increase in the size of the spleen.

Often in such patients there are pigment stones in the biliary tract. The only treatment is splenectomy.

Ovalocytic anemia is characterized by the presence of ovalocytes in the blood.

Anemia is inherited in an autosomal dominant manner, and its pathogenesis is due to a defect in the erythrocyte membrane. Acanthocytes (acanthus, thorns) are red blood cells that have 5-10 long, narrow, spike-like projections on their surface. In the membrane of such erythrocytes, the content of phospholipids is changed. When stomatocytosis red blood cells have in the center of the enlightenment in the form of the mouth. The clinic of these anemias is similar to the previous one.

Enzymopenic anemia differs in pathogenesis, and their clinical picture is similar.

Associated with violation of the structure and synthesis of hemoglobin. After the first report of Pauling in 1949 about an unusual hemoglobin in a patient with sickle cell anemia, more than 200 different variants of abnormal hemoglobins, that is, those in which the sequence of amino acids in the hemoglobin chains was disturbed, were described.

The first hemoglobin nomenclature was developed in 1953 by the International Symposium held by the US National Institutes of Health.

The usual type of hemoglobin of a healthy adult was marked with the letter “A” from the word adult (adult), “F” - fetal type (from the word fetus - fetus), “P” - primitive (germ hemoglobin up to 12 weeks).

The hemoglobin of sickle erythrocytes was called hemoglobin S, from the word sickle - sickle. Then it was proposed to call the newly discovered abnormal hemoglobins in capital letters of the Latin alphabet. Soon all the letters of the alphabet were used, and it was decided to call them by the name of the city, laboratories, hospitals where they were found, or by the name of the sick.

Sickle cell anemia with HBS carriers is characterized by hemolytic crises, arthritis. The appearance of patients is characteristic: asthenic physique, long thin limbs, an increase in the size of the abdomen due to splenomegaly. Limb ulcers are quite common. In the blood, hemoglobin is reduced to 50-60 g / l, erythrocytes to 1.5-2.0х10.12 / l, sickle cell erythrocytes.

Thalassemia. The basis of their pathogenesis is hereditary decrease in the rate of synthesis of one of the normal chains of HB. Thus, major thalassemia or Cooley's disease is characterized by a clinic of severe progressive hemolytic anemia, which is detected by the end of the first year of life, which leads to a lag in the mental and physical development of the child, signs of "mongoloid" appear, severe pallor and yellowness of the skin and mucous membranes, ulcers on the legs, changes in the bone system. The peripheral blood contains target erythrocytes.
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Congenital hemolytic anemia

    For the first time, Thompson (1939), and subsequently Haden (1947), described a familial form of hemolytic disease, which differs from the classical microspherocytic form of the Minkowski – Chauffard by a number of clinical and hematological features, in particular, the absence of erythrocyte spherocytosis (hence the name of this form is “non-spherocytic” hemolytic anemia), The disease is congenital, inherited by
    Acute hemolytic processes, accompanied by severe anemia and jaundice, occur in case of poisoning by hemolytic poisons of organic and inorganic origin (snake venom, fungal poison, saponins, arsenic hydrogen, phosphorus, phenylhydrazine), in severe burns or due to septic, streptococcal or anchoic anaemia or anemia. . Hemolytic anemia of infectious nature
    Acquired hemolytic anemias include: • immune hemolytic anemia; • drug hemolytic anemia; • traumatic and microangiopathic hemolytic anemia; • anemia caused by hemolytic poisons, chemicals, bacterial toxins; • paroxysmal night
  5. Hemolytic anemia
    A common sign of hemolytic anemia is the increased destruction of red blood cells, causing on the one hand anemia and increased formation of degradation products, on the other - reactively enhanced erythropoiesis. An increase in the decomposition products of erythrocytes is clinically manifested by lemon jaundice, a high content of unbound (unconjugated) bilirubin in the blood, with its
  6. Hemolytic anemia
    Hemolytic anemias make up an extensive group of anemic states of various etiologies, a common feature of which is the pathologically increased destruction of red blood cells. It is known that the average lifespan of erythrocytes does not exceed 100-120 days. Hemolytic anemia is characterized by shortening the life span of red blood cells. Hemolysis can occur intracellularly or
  7. Acquired hemolytic anemia
    This is a heterogeneous group of hemolytic anemias that occur under the influence of a number of factors that have the ability to damage red blood cells and cause their premature destruction. Classification of acquired hemolytic anemia [Idelson LI, 1975] I. Hemolytic anemia associated with exposure to antibodies. 1. Isoimmune hemolytic anemia 1) hemolytic disease of the newborn
  8. Hemolytic anemia
    Hemolytic anemias include an extensive group of anemic conditions that occur when blood destruction processes predominate over blood formation. The clinical picture of hemolytic anemia is very variable and depends both on the nature of the hemolytic agent and the duration of its effects on the body, and on the degree of compensation of the hemolytic process. Classification.
    Hemolytic anemia is a group of anemias characterized by hemolysis syndrome - increased red blood cell destruction. Red blood cells live for about 120 days, then they are destroyed - phagocytosed by macrophages, mainly the spleen. With hemolytic anemia, the life span of red blood cells is significantly shortened, and when it is reduced below 30 days, a hemolytic clinic appears
    The following groups of hereditary hemolytic anemias are distinguished: 1. Caused by violation of the structure of the erythrocyte membrane. 2. Associated with the violation of the activity of erythrocyte enzymes. 3. Associated with the violation of the structure or synthesis of hemoglobin. Hereditary hemolytic anemia due to erythrocyte membrane disorder Hereditary hemolytic anemia associated with
  11. Hemolytic anemia
    DEFINITION Regardless of the cause, a fundamental characteristic of any hemolytic anemia is a reduction in the life span of red blood cells. ETIOLOGY Hemolytic anemias are often noted in the neonatal period, and they can be caused by various factors, internal and external to red blood cells. Hemolysis can be caused by three main groups of causes:
  12. Acquired hemolytic anemia
    1. Immunohemolytic anemia a) autoimmune b) isoimmune 2. Acquired membranopathies a) paroxysmal nocturnal hemoglobinuria b) spore cell anemia 3. Associated with mechanical damage of erythrocytes a) march hemoglobinuria b) Moschkovich disease (microangiopathic thrombocytes)
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