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Hereditary and congenital hemolytic anemia in children and adults

Class of anemiaSubtypes of anemiaBrief description of hemolytic anemia
Hereditary hemolytic anemiaAsterocampa hemolytic anemiaAccelerated destruction of red blood cells caused by insufficient activity of enzymes, which are responsible for maintaining their life cycle. This files most often have to deal with hereditary hemolysis in the enzyme deficiency glucose-6-phosphate dehydrogenase. Transmitted either through the defect in the X chromosome or an autosomal recessive way (is not in all cases).
Microperimetry hemolytic anemia of Minkowski'sThe disease occurs in connection with the occurrence of mutations or transfer of the mutated genes responsible for synthesis of specific protein molecules and proteins that form the wall of the red blood cells. This breaks their structure, functional activity and resistance to destruction in the form of a sharp reduction in size and shortening of the circulation in the vascular space.
Sickle cell anemiaHereditary hemolysis due to a mutation in the gene that encodes the sequence of amino acids in the synthesis of hemoglobin. This leads to an abnormal S-shape of hemoglobin, which distorts the erythrocyte into a sickle. These cells are not able to change their form in the circulation in the capillary vessels, which causes their increased destruction.
TalasemiaThis group of hereditary hemolytic anemias caused by disorders of the process of formation of normal hemoglobin. However, he loses some of the protein chains, causing premature oxidation. The resulting products of peroxidation destroys membrane of red blood cells that lies at the basis of their hemolysis.
Acquired hemolytic anemiaAutoimmune hemolytic anemiaThe mechanism of hemolysis of erythrocytes caused by formation of antibodies which are deposited on the surface of their membranes. As a result of erythrocyte cells acquire specific labeling and, therefore, are perceived as foreign by macrophages. As a result, the destruction of red blood cells by the cells own immune system, causing a decrease in their quantity and anemia.
Anemia hemolytic disease of the newborn and RH-conflictOne of the varieties with the immune mechanism of development. Most often caused by RH incompatibility of blood of mother and fetus. At the same time inRH-negative mother's body formed antibodies to the fetal red blood cells containing on the surface of the RH antigen. As a result, the formation of immune complexes that destroy the erythrocytic cells.
Traumatic acquired hemolytic anemiaThe destruction of normal erythrocytes in the vascular space in the process of their circulation. The cause of the disease can become artificial vascular prostheses and heart valves, atherosclerotic lesions and anomalies of the aorta and major blood vessels, disruption of the structure of capillaries and other elements of the microvasculature.
Hemolysis due to the influence of external and internal factorsThe causes of the disease hidden in the destruction of normal red blood cells under the influence of drugs, viruses, and other industrial poisons, and various toxic compounds and intoxications on the background of serious infection or critical illness.
Acute paroxysmal nocturnal hemoglobinuriaA rare disease, manifested attacks of massive destruction of red blood cells. The basis of this type of hemolytic anemia is the formation of a small defect in the membrane of erythrocytes under the action of the proteins of the complement system (immune blood proteins).

Additionally, patients assigned this diagnosis:

  1. Evidence of hemolysis (considered only two options: hemolysis + or -;
  2. Urinalysis: detects the presence of haemoglobin in urine and increased concentrations of urobilin;
  3. Biochemical study of blood. Determines the higher concentration of bilirubin, more indirect;
  4. Bone marrow puncture with the definition of myelogram. Determined by the stimulation of red sprout blood.

Treatment options

Treatment of hemolytic anemia is much more difficult than any others. This is due to the fact that the impact on the launch mechanism of hemolysis is not always possible. The overall plan of therapeutic measures may include:

  1. Application of preparations of folic acid and vitamin b12;
  2. The transfusion of washed erythrocytes. Shown in the decline in red blood up critical numbers;
  3. Introduction glucocorticoid hormones (dexamethasone, prednisone, methylprednisolone, Cortine);
  4. Transfusion of fresh frozen plasma and human immunoglobulin;
  5. Prevention of infectious complications and exacerbations of existing chronic diseases;
  6. The use of cytostatics. Shown exclusively in autoimmune hemolytic anemia;
  7. Surgical treatment in the form of a splenectomy (removal of spleen). Method providesrelatively good prognosis for recovery. Not effective in anemia Minkowski-of's and other types of hereditary hemolysis.

Any hemolytic anemia, the struggle which started late is a difficult problem. It is unacceptable to try to handle it by yourself. Its treatment should be integrated and assigned exclusively by a qualified technician on the basis of a thorough examination of the patient.