Anemia (anemia)

Anemia (anemia) - a decrease in blood total hemoglobin, which, with the exception of acute blood loss, is characterized by a decrease in hemoglobin in a unit of blood volume. In most cases, the level of erythrocytes in the blood decreases with anemia. However, in iron deficiency states, anemia associated with impaired synthesis of porphyrins, thalassemia, the erythrocyte content in the blood can remain normal (with thalassemia - often elevated) with a low level of hemoglobin. In acute hemorrhages (massive bleeding or acute hemolysis) in the first hours, the level of hemoglobin and erythrocytes in the blood remains normal, although there are obvious signs of anemia-the pallor of the skin, conjunctiva, veins, palpitations and shortness of breath at low loads, and in severe cases Blood pressure.

Anemia is always secondary, i.e., one of the symptoms of some common disease. Along with the frequently occurring and easily diagnosed forms of anemia, there are very rare anemia syndromes that require complex diagnostic techniques to diagnose. Some forms of anemia can be diagnosed only in specialized institutions.

Anemia posthemorrhagic acute - anemia due to acute blood loss within a short period of time. The minimal blood loss, which poses a danger to the health of an adult, is 500 ml.

Etiology. The cause of hemorrhage can be trauma, surgical intervention, bleeding in case of ulcer of the stomach or duodenum, from esophageal veins, in violation of hemostasis, ectopic pregnancy, lung diseases, etc.

Pathogenesis consists of the phenomena of acute vascular insufficiency, caused primarily by the acute devastation of the vascular bed, the loss of plasma; With massive blood loss, hypoxia also occurs due to the loss of red blood cells, when this loss can no longer be compensated by the acceleration of circulation due to the increase in cardiac contractions.

Clinical picture. Vascular disorders: palpitation, shortness of breath, falling arterial and venous pressure, pallor of the skin and mucous membranes. The severity of these changes is not in a clear relationship only with the degree of hemorrhage, as often the collapse also appears in response to pain, a trauma that caused blood loss. A significant sign of internal bleeding is sudden dryness in the mouth. The severity of the clinical picture is determined not only by the amount of blood lost, but by the rate of hemorrhage. Of particular importance is the source of blood loss: bleeding into the gastrointestinal tract may be accompanied by an increase in body temperature, a picture of intoxication, an increase in the level of urea in the blood (at a normal level of creatinine); Cavitary bleedings can even cause a small amount of bleeding to cause symptoms of compression of the organs, etc.

Treatment begins with stopping bleeding. Rapid reduction of hemoglobin content below 80 g / l (8 g%) of hematocrit below 25%, plasma proteins below 50 g / l (5 g%) is the basis for transfusion therapy, with the loss of erythrocytes sufficient to replenish by 1/3; The primary task is to restore the volume of circulating blood by transfusing colloidal solutions (albumin placental or blood, fresh frozen plasma or protein), polyglucin or gelatin. In the absence of polyglucin and gelatin, 1000 ml of a 10% solution of glucose can be poured, followed by 500 ml of a 5% solution; Glucose is poured at a rate of 0.5 g / (kg-h). Rheopolyglucin and its analogues as desaggregants (i.e., preparations that reduce blood clotting capacity) should not be used in conditions of possible resumption (or ongoing) of bleeding. To compensate for the loss of red blood cells, the erythrocyte mass is used. The indication for red blood cell transfusion is the apparent massiveness of blood loss (more than 1 L), the preservation of pallor of the skin and mucous membranes, tachycardia and dyspnea, despite the restoration of the volume of circulating blood with the help of plasma substitutes.

In no case should one strive to replenish blood loss through erythrocytes "drop by drop"; Freshly frozen plasma together with plasma substitutes must be poured in a larger volume than was lost, and erythrocytes - significantly less. The resulting hemodilution results in an improvement in microcirculation, while massive transfusions of whole blood or erythrocytes increase the formation of microstatic cells due to shock, contribute to the development of DIC syndrome.

Small blood loss is supplemented by the introduction of saline solutions, albumin. Whole blood is poured in the absence of erythrocyte mass, and if there is no preserved blood, freshly citrated blood is poured (warm, freshly prepared) or direct transfusion (very undesirable without extreme necessity due to the presence of microbots in such blood). In case of surgical bloody cracks less than 1 l, transfusion of erythrocyte mass is usually not used.

Anemia iron deficiency is associated with iron deficiency in the body, leading initially to multiple trophic disorders (dry skin, brittle nails, hair loss), as the function of tissue respiratory enzymes containing iron worsens, and then hemoglobin formation breaks down, hypochromic anemia develops.

Etiology, pathogenesis is determined by inadequate intake of iron: in most cases, the crucial role played by chronic blood loss, not compensated by iron intake from food, less often (it happens in children) anemia is caused by a small initial intake of iron in the fetus due to iron deficiency in the mother. A special group consists of patients after resection or with severe damage to the small intestine (resection of the stomach without concomitant enteritis does not lead to the development of iron deficiency).

Clinical picture. Lethargy, increased fatigue even before the development of severe anemia, constipation, headache, perversion of taste (patients eat chalk, clay, appears, a tendency to acute, salty foods, etc.), fragility, curvature and transverse striation of nails, hair loss , Imperative urge to urinate, urinary incontinence. In addition to these signs that are not directly related to the level of hemoglobin in the blood, signs specific for all anemia are associated with the degree of anemia: pallor of the skin and mucous membranes, palpitation, shortness of breath during physical exertion. Important in the clinical picture is the nature of the disease that determined iron deficiency (gastric ulcer, duodenal ulcer, hemorrhoids, uterine fibroids, abundant menorrhagia, etc.). Anemia is detected when examining the blood. The color index is usually low (sometimes below 0.5); Pronounced hypochromia of erythrocytes, ishanocytes, poikilocytosis; ESR is usually slightly increased. The most important indicator of the disease is a decrease in the serum iron level (the norm is 13-28 mmol / L for men, 11-26 mmol / L for women). Check it can be no less than a week after the abolition of preparations containing iron.

The diagnosis of iron deficiency anemia is preliminarily established with a reduced level of hemoglobin and a low color index against the background of trophic disorders typical for iron deficiency states. It becomes evident with a low level of serum iron and ferritin, as well as an increase in the level of total iron binding capacity of the serum. But even in this case, it is required to establish precisely the cause of iron deficiency, after which the diagnosis of the disease as a whole is formed. With a low color index, but without lowering the serum iron level and without trophic disorders typical of iron deficiency, thalassemia and anemia caused by impaired synthesis of porphyrins, in particular lead intoxication, occur.

Blood loss from the gastrointestinal tract is one of the common causes of iron deficiency anemia. Gastrointestinal hemorrhage can remain hidden for many years, despite multiple radiographic, gastro, duodeno- and colonoscopy studies, since fibroids and small intestinal angiomas are not always detected in these studies. Quantitatively, blood loss from the gastrointestinal tract can be assessed only with the help of a label of erythrocytes in 51Cr.

Another hidden cause of hemorrhage can be hemorrhages in the pulmonary parenchyma with isolated pulmonary hemosiderosis. The disease is characterized by relapsing episodes of pneumonia with the development of iron deficiency anemia, accompanied by a periodic rise in the level of reticulocytes in the blood. Radiography reveals cloudy multiple, often asymmetric shadows, which can disappear within 1-2 days. The physical data are very scarce: a somewhat exhaled breath, individual crepitating rales are heard. Body temperature is often low. Shadowformations in the lungs are caused by hemorrhages (if their volume is large and they repeat, then iron deficiency anemia develops), accompanied by the appearance in the sputum of "heart defect cells" - macrophages saturated with hemosiderin. Repeated pulmonary hemorrhage can lead to the development of pulmonary hemosiderosis. In doubtful cases, a biopsy of the lungs is necessary for diagnosis.

The described picture of pulmonary hemosiderosis in combination with a severe progressive nephritis - Goodpasture's syndrome - can also be accompanied by a coronary artery anemia. The Goodpasture syndrome develops as a result of the formation of autoantibodies to the basal membrane of the glomeruli of the kidneys; The same antibodies also act on the basal membrane of the pulmonary alveoli. A similar clinical picture that develops on glomerulonephritis and hemoptysis affords endothelial damage to the glomeruli of the kidneys and lung vessels with immune complexes that can appear in response to an infectious process of any nature and are determined by immunofluorescence in a biopsy of the kidney or lung tissue. Immune complexes are often represented by cryoglobulins (a complex of immunoglobulins precipitating at a temperature below 37 ° C) that can circulate in the blood, deposited under the endothelium of the vessels of different organs and tissues, causing the development of generalized vasculitis, for example, Shenlaine-Henoch disease. Iron deficiency anemia can be a consequence of a chronic inflammatory process in the body (chronic pyelitis, sepsis, etc.).

The hidden source of blood loss can be so-called chocolate cysts. Often we are talking about ovarian cysts, repeated hemorrhages in which lead to a deficiency of iron in the body, since hemosiderin formed in places of hemorrhage is not absorbed and is not disposed of for the needs of hematopoiesis. These cysts are recognized with a thorough gynecological and radiological study, using computed tomography, ultrasound. Insufficient intake of iron from food can lead to iron deficiency anemia in pregnancy and lactation, increased growth during puberty in girls, although in all of these cases there is usually a hidden, previous iron deficiency. Iron deficiency anemia May be in premature infants, in children from multiple pregnancies. Menorrhagia and metrorrhagia (for example, with uterine myomas), hemorrhoids hemorrhoids, nosebleeds often cause anemia. However, in all cases of seemingly obvious causes of iron deficiency anemia, it is necessary to exclude (especially clinically) the possible other sources of blood loss in the most careful way, since anemia is often caused not by one but several reasons. The appearance of iron deficiency anemia in the elderly regardless of sex requires the exclusion of a possible swelling of the gastrointestinal tract.

Treatment. First of all, it is necessary to eliminate the cause of blood loss. Necessarily prescribe iron preparations: gemostimulin, ferrocal, ferroplex, orferon, konferon. In severe forms of anemia it is recommended to take 2 tablets of one of these drugs 3 times daily before meals. In case of poor tolerance (nausea, abdominal pain), the tablets are taken after or during meals and in such quantity that dyspeptic disorders are not present. Reducing the dose slows the normalization of the blood composition. Iron preparations are usually taken for several months, with a persistent source of blood loss (eg, menorrathy), they are prescribed with small interruptions almost continuously, focusing primarily on subjective signs of iron deficiency (increased fatigue, drowsiness), the appearance of brittleness and deformation of the nails, dry skin , And then on the blood counts, which, with a properly organized dispensary observation of such patients, should be normal.

The intake of iron preparations enhances the bone-marrow production of erythrocytes and is accompanied by an increase in the level of reticulocytes in the blood 8-12 days after the initiation of therapy (a fact having differential diagnostic significance). Assign hydrochloric acid, B vitamins along with iron preparations are not necessary; Somewhat enhances the absorption of iron ascorbic acid. Treatment with parenterally administered iron preparations is less effective than taking it in the form of tablets inside.

For intramuscular administration, use is made of the gum, ferbitol, Ferrum Lek, etc. The latter preparation is also produced for intravenous administration. Indications for parenteral administration of iron in iron deficiency anemia are severe enteritis, conditions after extensive resections of the small intestine (but not gastrectomy). Doses are prescribed at the rate of 1.5 mg of iron per 1 kg of body weight per day. The zhetofer is injected in / m 2 ml per day to a patient with a body weight of 60 kg.

With isolated pulmonary hemosiderosis, along with treatment with iron preparations, immunosuppressive therapy - prednisolone - is used, and if it is ineffective and the clinical condition is severe, azathioprine (imarant), cyclo-phosphan or other cytotoxic drugs. In the Goodpasture Syndrome, the same immunosuppressive agents are used for therapeutic purposes, along with plasmapheresis, aimed at removing immune complexes.

Zhadezodefitsitnaya anemia, caused by the infectious process, requires primarily active antibacterial therapy. Iron preparations in an acute process should not be prescribed if its deficiency is due only to infection.

Treatment with iron preparations is usually performed on an outpatient basis. Control blood tests are advisable after 1.5 weeks from the start of treatment to detect reticulocyte recovery and in a month when the hemoglobin level usually approaches normal; at. Further control is sufficient to carry out 1 time in 3 months and even less often if the patient well assesses the subjective signs of iron deficiency. These indicators are an important control factor for the systematic prevention of iron deficiency conditions. Even in the absence of obvious signs of iron deficiency, but the identification of certain of its symptoms without the development of anemia in pregnant women and women suffering from profuse menorrhagia, it is advisable to appoint 1 tablet of iron per day before the elimination of all these phenomena (vitamin B12 and other B vitamins in such situations are useless !). Often observed in pregnancy pseudoanemia due to hydremia (hemoglobin about 85-100 g / l, color index close to unity, pink complexion and mucous membranes, absence of pronounced anisocytosis of erythrocytes) does not require any special anti-anemic treatment. Pseudoanemia completely passes by itself.

Transfusion of blood with iron deficiency anemia is not indicated, except for severe conditions associated with massive blood loss and accompanied by signs of acute posthemorrhagic anemia.

Iron deficiency anemia in the elderly often requires the connection and cardiac funds, as it is complicated by heart failure, fluid retention, hydremia, which in itself worsens the blood test. Evidence of hydromic pseudoanemia is an increase in red blood after treatment with diuretics and cardiotonic drugs.

Hereditary anemia associated with impaired synthesis of porphyrins (sideroachrestic anemia), characterized by hypochromia of erythrocytes, increased serum iron levels, deposition of iron with a picture of hemoscaderosis of organs.

Pathogenesis of the most frequent form is associated with a disruption in the synthesis of protoporphyrin, which leads to a disruption in the binding of iron and its accumulation in the body with a severely impaired formation of hemoglobin.

Clinical picture. Moderate anemia with a sharply reduced color index. Trophic disorders characteristic of iron-deficient states are not observed. There are no complaints or slight weakness, fatigue. Most often men suffer. Disease is inherited by a recessive type, inheritance is linked to the X chromosome. At the beginning of the disease, there is sometimes a slight increase in the spleen, in the future, in connection with increased deposition of iron in the organs, liver hemosiderosis develops (the liver increases and becomes dense), the pancreas (a picture of diabetes mellitus appears). The accumulation of iron in the cardiac muscle leads to severe circulatory insufficiency, hemosiderosis of the testicles is accompanied by the development of eunuchoidism. Sometimes the skin becomes gray.

Blood picture: pronounced red blood cell hypochromia (color index 0.4-0.6), hemoglobin level is lowered, the number of erythrocytes is less changed, serum iron level is increased. Morphologically marked anisocytosis, poikilocytosis, sometimes a small number of target forms. The content of reticulocytes is usually normal. Other blood counts are normal.

The diagnosis is established on the basis of hypochromic anemia without any signs of iron deficiency syndrome (there is no pronounced asthenia, dry skin, hair loss, fragility and deformation of the nails, etc.), elevated serum iron level. It should be borne in mind that iron deficiency anemia is more common in women, and this form is almost exclusively in men. When studying the bone marrow, in contrast to iron deficiency anemia, a high percentage of ringed sideroblasts is noted (almost all erythrocaryocytes-ring sideroblasts). Differential diagnosis with thalassemia - see Thalassemia. Finally, a diagnosis can be made when examining the level of porphyrins in erythrocytes.

Treatment. Assign pyridoxine (vitamin B6) no 1 ml of a 5% solution v / m 2 times a day. With effective therapy after 1.5 weeks, the content of reticulocytes sharply increases, and then the level of hemoglobin also increases. When the activity of the pyridoxal kinase enzyme is disrupted, the effect results in the use of coenzyme of vitamin B6-pyridoxal phosphate (2 mg 3 times daily in tablets). For the treatment of hemosiderosis as such, accompanied by pathology of the liver, pancreas and other organs, as well as for the treatment of forms resistant to vitamin B12 (they are affected by both men and women, the inheritance is reciprocal, not linked to the sex), deferoxamine (desferal) Courses for at least a month at 500 mg IM daily. Such courses can be 4-6 per year. Increased excretion of iron from the body with deferoxamine not only contributes to the normalization of functions impaired by hemosiderosis of organs, but also reduces the degree of anemia, improving the activity of bone marrow blocked by excess iron.

Anemia in lead poisoning is caused by impaired synthesis of porphyrins and gemma

Pathogenesis is determined by the blockade of the enzymes involved in the synthesis of heme. As a result, hypochromic anemia develops with a high level of serum iron. In addition, lead damages the envelope of erythrocytes, causing increased hemolysis. As a result of the violation of heme synthesis, the excretion of porphyrins from the body increases, and, which is pathognomonic for lead poisoning, delta-aminolevulinic acid, the level of which in the urine rises tenfold.

The clinical picture consists of common signs of anemic syndrome without the phenomena of iron deficiency, polyneuritis, paroxysmal pain in the abdomen. Blood test reveals hypochromic anemia, an increase in the content of reticulocytes to 10% due to hemolysis, basophilic puncture in erythrocytes, a high level of serum iron. In the bone marrow a high percentage of sideroblasts. The diagnosis is confirmed by a significant increase in urine of delta-aminolevulinic acid. Another important sign of lead poisoning is an increased release of lead in the urine when complexons are introduced into the body (tetacin-calcium, oxathiol, etc.). Some help in diagnosis is given by anamnestic information about contact with lead (for example, casting shot, toys, milk or pickled vegetables stored in clay crockery, glaze which often contains lead).

Treatment is carried out with complexons under the control of the level of lead and delta-aminolevulinic acid in the urine. With effective therapy, its content in the urine is normalized.

Megaloblastic anemia is a group of anemia, the common feature of which is the detection in the bone marrow of peculiar erythrocaryocytes with structural nuclei that retain these features in the late stages of differentiation (the result of a violation of DNA and RNA synthesis in cells called megaloblasts), in most cases megaloblastic anemia is characterized by a color index higher Units. Since the synthesis of nucleic acids affects all bone marrow cells, frequent signs of the disease are a decrease in the number of platelets, leukocytes, an increase in the number of segments in the granulocyte.

Anemias associated with vitamin B12 deficiency, regardless of the causes of this deficiency, are characterized by the appearance of megaloblasts in the bone marrow, intracranocerebral disruption of erythrocytes, macrocytic anemia, thrombocytopenia and neutropenia, atrophic changes in the mucous membrane of the gastrointestinal tract and changes in the nervous system in the form of funicular myelosis.

Etiology, pathogenesis. The idiopathic form of B12-deficient anemia (pernicious anemia) develops as a result of inadequate intake of exogenous cyanocobalamin (vitamin B12), which occurs mainly in the elderly. The pathogenesis of vitamin B12 deficiency is more often associated with impaired glycoprotein production, combining with food vitamin B12 and ensuring its absorption (internal factor). Often the first signs of the disease appear after the transferred enteritis, hepatitis. In the first case, this is associated with a violation of absorption of vitamin B, in the small intestine, in the second - with the consumption of its reserves in the liver, which is the main depot of vitamin B12. However, both these and other provocative moments can play the role of a trigger mechanism only with the previously hidden hidden deficiency of this vitamin due to a violation of the secretion of the internal factor. Development of B12-deficiency anemia after total gastrectomy (when the secretion of the internal factor is completely eliminated) occurs 5-8 years or more after the operation. During this period, patients live with vitamin stores in the liver with a minimal replenishment of it due to insignificant absorption in the small intestine not connected with the internal factor of the vitamin.

A very rare form of B-deficiency anemia is a violation of vitamin assimilation during invasion by a wide ribbon, when a parasite absorbs a large amount of vitamin B12. The causes of impaired absorption of vitamin B12 in intestinal lesions can be severe chronic enteritis, terminal ileitis, diverticulosis of the small intestine, the appearance of a blind loop of the small intestine after surgery on it.

In the emergence of vitamin deficiency in recent cases, its absorption by the excessively developed intestinal microbial flora plays an important role.

A rare form of vitamin B12 deficiency is the recessively inherited syndrome of Imerslund-Gresbek with epithelial damage to the intestine, kidneys, and skin. The disease develops in children and is characterized by a combination of megaloblastic anemia with kidney damage. Occasionally, the disease happens in adults.

One of the reasons for the impaired isolation of the internal factor can be chronic alcohol intoxication, when it is accompanied by toxic damage to the gastric mucosa. The cause of the most common idiopathic form of the disease is not entirely clear, although for many cases hereditary nature (recessive inheritance) can be proved.

Symptoms, course, diagnosis. The clinical picture of vitamin B12 deficiency is characterized by damage to the hemopoietic tissue, digestive and nervous systems. Weakness, fatigue, palpitation with physical activity are noted. One of the characteristic features is a funicular myelosis. Gastric secretion is inhibited, persistent achlorhydria is possible. Often there are signs of glossitis - a "polished" language, a burning sensation in it. The skin of patients is sometimes slightly yellown, the serum level of indirect bilirubin is increased (due to increased death of hemoglobin-containing bone marrow megaloblasts). A small increase in the spleen is determined, less often the liver.

Blood picture: pronounced anisocytosis of erythrocytes along with very large (more than 12 microns in diameter) megalocyte cells, acute poikilocytosis, increased saturation of erythrocytes with hemoglobin-hyperchromia, the appearance of polysegmented neutrophils, hyperchromic, less often normochromic nature of anemia, thrombocytopenia, and leukopenia. The degree of cytopenia can be different, and there is rarely a parallelism in reducing the level of all three blood cells. Of decisive importance in the diagnosis belongs to the study of bone marrow, which reveals a sharp increase in the number of elements of the erythroid series with a predominance of megaloblasts. The described picture refers to distant states.

Often B12-deficiency anemia is determined only by the pattern of blood in patients without any complaints: moderate, usually hyperchromic anemia, leukopenia, thrombocytopenia (sometimes either thrombocytopenia or leukopenia). Polysegmentation of neutrophil nuclei, marked changes in the shape of red blood cells may be absent. In all cases, a large percentage of megaloblasts are found in the bone marrow. However, if the patient received a vitamin B12 injection several days before the bone marrow puncture, the bone marrow metastases may not be very pronounced, or absent altogether. Consequently, in all cases of normochromic or hyperchromic anemia accompanied by leuko- and thrombocytopenia (a non-mandatory sign), one must remember the most important rule: to perform a bone marrow puncture before the appointment of vitamin B.

Cells of the red row, very reminiscent of megaloblasts, can occur in acute erythromyelosis, which, like B12-deficiency anemia, is accompanied by a slight icterus, often combined with leuko- and thrombocytopenia. However, with this leukemia there is no such pronounced aniso- and poikilocytosis as in B12-deficient anemia, and most importantly, in the bone marrow, along with megaloblast-like cells, myeloblasts or undifferentiated blasts occur in large numbers. In doubtful cases, after bone marrow puncture, start therapy with vitamin B12, which in B12-deficient anemia after 8-10 days will lead to a sharp increase in the percentage of reticulocytes in the blood (recyculocyte crisis), a rise in hemoglobin level, disappearance of pronounced anisocytosis in the blood, and in bone The brain - megaloblasts. With acute erythromyelosis, the introduction of vitamin B12 can not change either the blood picture or the patient's condition.

Determining the cause of vitamin B12 deficiency is the next stage of diagnosis. It should be borne in mind that sometimes a vitamin B12 deficiency is combined with stomach cancer, since stomach cancer is more likely to occur in people suffering from this form of anemia. Along with carrying out all the obligatory studies (for the prevention of invasion by a wide ribbon, enteritis, etc.) it is necessary to be convinced by gastrocopia or X-ray study in the absence of stomach cancer.

Treatment. Cyanocobalamin (vitamin B12) is administered daily s / c at a dose of 200-500 mgk once a day for 4-6 weeks. After normalization of hemopoiesis and blood composition, coming in 1,5-2 months, the vitamin is administered once a week for 2-3 months, then for half a year 2 times a month (in the same doses as at the beginning of the course). In the future, patients should be placed on dispensary records; Prophylactically they are administered vitamin B12 1-2 times a year in short courses of 5-6 injections. This recommendation can be changed depending on the dynamics of blood parameters, the state of the intestine, and liver function.

Diagnosis of the disease, the most complex element of which is bone marrow puncture, and treatment with vitamin B ^ can be carried out on an outpatient basis. Duration of disability is determined by the severity of anemia and neurological disorders. There is practically no permanent disability with this disease. A dangerous, life-threatening complication of vitamin B deficiency is coma development. More often this condition is observed in the deep old age of the streets, which long before this complication they examined peripheral blood or did not check blood at all for dozens of years. Old people who are in a comatose state, after establishing a low hemoglobin level, should immediately perform a sternal puncture, stain the smear and, upon seeing a picture of B12-deficient megaloblastic anemia, begin the introduction of large doses of the vitamin; Transfusion of erythrocyte mass begins only when a low level of hemoglobin is detected.

Fapievo-scarce anemia-megaloblastic anemia, similar in basic manifestations with B12-deficiency anemia; Develops with a deficiency of folic acid. Violation of absorption of folic acid is observed in pregnant women suffering from hemolytic anemia or abusing alcohol before pregnancy, in preterm infants, especially when they are fed by goat's milk, in individuals who underwent resection of the small intestine, in tropical sprue and celiac disease, in alcoholics, Reception of anticonvulsants such as phenobarbital, diphenine. The clinical picture, the picture of blood and bone marrow are similar to those with a deficiency of vitamin B12, but there is usually no glossitis and funicular myelosis.

The diagnosis of folate-deficiency anemia can be reliably established by reducing the level of folic acid in red blood cells and serum. In practical work, this is difficult to achieve. Detection of the megaloblastic nature of anemia in the conditions described above is a sufficient basis for the appointment of folic acid at a dose of 5-15 mg / day inwards (this dose provides a therapeutic effect even after resection of the small intestine, with enteritis, etc.). Retikulotsitarny recovery after 1.5-2 weeks of treatment indicates a correct diagnosis. Prevention of folic-deficiency anemia is to prescribe folic acid to people who have suffered a deficit once and who suffer from one of the conditions described above. Folic acid should be administered to pregnant women suffering from hemolytic anemia.

In addition to the described, recessively inherited forms of megaloblastic anemia occur, when anemia along with other symptoms occurs in children and can not be treated with vitamin B12 and folic acid. These patients should be examined in specialized institutions.

Hemolytic anemia is associated with increased destruction of erythrocytes. All hemolytic states are characterized by an increase in the blood content of the products of erythrocyte decay - bilirubin or free hemoglobin or the appearance of hemosiderin in the urine. An important sign is a significant increase in the percentage of "newborn" erythrocytes - reticulocytes in blood by increasing the production of red blood cells. Bone marrow in hemolytic anemia is characterized by a significant increase in the number of cells in the red row.

Hereditary microspherocytosis. Etiology, pathogenesis. Disease is inherited by the dominant type. The basis of hemolysis is a defect in the structure of the erythrocyte membrane, which causes its increased permeability and promotes the penetration of excess sodium into the erythrocytes and the accumulation of water. Erythrocytes acquire a spherical shape, damaged when passing through the narrow spaces of the spleen sinuses. Damaged cells are captured by macrophages (intracellular hemolysis); The conversion of hemoglobin into bilirubin leads to indirect hyperbilirubinemia, jaundice.

Clinical picture. Intracellular decay of erythrocytes determines the clinical manifestations of the disease-jaundice, enlarged spleen, anemia, a tendency to form gallstones, characteristic morphological changes in erythrocytes, reticulocytosis. The first clinical manifestations of the disease can be at any age, although in reality it begins with birth. A very rare and nonspecific symptom of the disease is the formation of trophic ulcers on the legs. Long, from early childhood, the existing hemolysis is accompanied by bone marrow hyperplasia, which in turn leads to a violation of bone formation. There is a deformation of the jaws with an incorrect position of the teeth, high sky, protruding forehead, light microphthalmia, etc. The spleen is always palpable.

Blood picture: microspherocytosis of erythrocytes, high reticulocytosis, usually estimated at tens of percent, normochromic anemia of different severity, sometimes the number of erythrocytes and hemoglobin are normal. During the hemolytic crisis, neutrophilic leukocytosis is possible. Very abruptly there are so called aregenerator crises, when elevated hemolysis for several days is not accompanied by erythropoiesis; Reticulocytes disappear from the blood, anemia rapidly builds up, the bilirubin level falls. Spherocytosis of erythrocytes corresponds to their reduced osmotic resistance. The average diameter of red blood cells decreases, respectively, their thickness increases, so that in most cells no central clarification is visible, since the erythrocyte from the biconcave turns into a globular.

Diagnosis. Jaundice, enlarged spleen, spherocytosis of erythrocytes, their decreased osmotic resistance, high reticulocytosis, deformation of the facial skull and enlarged spleen make the diagnosis of hereditary spherocytosis unquestionable. Typically, similar symptoms can be found in one of the parents of the patient, although their severity may be different. In rare cases, the parents are perfectly healthy. Difficulties in diagnosis are often caused by cholelithiasis, usually accompanying hereditary microspherocytosis (due to the formation of bilirubin stones in the ducts and gall bladder). The inherent hemolysis indirect bilirubinemia with cholelithiasis is replaced by a straight line - there is mechanical jaundice. Soreness in the gallbladder area, some enlargement of the liver are common symptoms in hereditary microspherocytosis. Often, for many years, patients are mistakenly treated as those suffering from biliary tract or liver disease. One of the sources of errors is the lack of information about reticulocytes.

Spherocytosis of erythrocytes and other signs of hemolysis (jaundice, enlarged spleen, reticulocytosis) occur in autoimmune hemolytic anemia. However, unlike hereditary microspherocytosis, there are no changes in the facial skull, signs of hereditary microspherocytosis in any of the parents; At the first clinical manifestations of autoimmune hemolysis there is still no significant increase in the spleen, pain in the gallbladder, but anisocytosis and poikilocytosis of erythrocytes are more pronounced than with microspherocytosis. In doubtful cases, it is necessary to put a Coombs test, which is positive (direct sample) in most cases of autoimmune hemolytic anemia and negative in hereditary microspherocytosis. Characteristic for hereditary microspherocytosis changes are revealed in the acid erythrogram: a sharp increase in the resistance of erythrocytes to the action of hydrochloric acid with a typical curve of their lysis in time, a decrease in their resistance after washing.

Treatment. Radical method - splenectomy, shown with expressed hemolysis, anemia, cholelithiasis, trophic ulcers of the shin. In children it is desirable to have a splenectomy at the age of 7-8 years, but severe anemia, severe hemolytic crises are a direct indication for surgery at any age. After the operation, there is a practical recovery in all patients, although there remains a spherocytosis of erythrocytes and very small signs of elevated hemolysis. With calculous cholecystitis concurrent with splenectomy, cholecystectomy can be performed. At aregenerator crises transfuse erythrocyte mass, sometimes prescribe prednisolone in a dose of 40-60 mg per day.

Hereditary ovalocytosis (elliptocytosis) is the dominant hereditary anomaly of erythrocytes, sometimes complicated by elevated hemolysis. In most carriers, there is no abnormality of signs of elevated hemolysis. The clinical picture of the disease in hemolysis corresponds to hereditary microspherocytosis. The diagnosis is established on the basis of ovalocytosis of most erythrocytes and signs of intracellular hemolysis (jaundice, reticulocytosis, enlarged spleen).

Treatment. With expressed hemolysis, splenectomy. Hereditary dentocytosis-dominant inherited anomaly of the form of erythrocytes, sometimes complicated by intracellular hemolysis. The anomaly itself is asymptomatic. The pathogenesis of the hemolytic syndrome seems to be similar to that of hereditary spherocytosis. The clinical picture of hemolytic syndrome is characterized by jaundice, reticulocytosis, enlarged spleen. The specific feature of red blood cells in this disease is two peculiar lines joining the edges of the cell in the region of central erythrocyte bleaching. They give it the shape of the mouth, hence the name of the anomaly.

Treatment. With expressed hemolytic syndrome - splenectomy.

Hemolytic anemia due to deficiency of glucose-6-phosphate dehydrogenase (G-6-FDH). The disease is widespread in several countries in Asia and Africa. In the USSR it is found in Azerbaijan, less often in other republics of Transcaucasia, sporadically - everywhere. The gene regulating the synthesis of G-6-FDH is located in the X chromosome. Disturbances in the structure of this gene, leading to a deficiency of the enzyme, are found predominantly in men. Genetic disorders in this structural gene vary quite widely: the activity of the enzyme in some cases is only slightly lower than normal, in others it is almost nonexistent. Very diverse pathological processes associated with a deficiency of G-6-FDH. Clinically, they manifest hemolytic crises in response to taking certain medicines, eating horse beans, but a chronic hemolytic process is also possible, exacerbated by the same provoking effects.

The pathogenesis of elevated hemolysis with deficiency of G-6-FDH is not entirely clear. Violation of the recovery processes in the erythrocyte leads to the oxidation of hemoglobin, the precipitation of globin chains, the increased destruction of such erythrocytes in the spleen.

Clinical picture. Hemolytic crisis with G-6-FDG deficiency is characterized by the appearance of dark urine (hemosiderin and free hemoglobin in the urine), a slight jaundice on the 2-3rd day of taking a medication that provoked hemolysis. Further administration of this medication leads to severe intravascular hemolysis with an increase in body temperature, pain in the bones of the hands and feet, in severe cases - the fall of blood pressure, anemic coma. In the blood-neutrophilic leukocytosis (sometimes very high) with a shift to myelocytes, severe anemia, high reticulocytosis. Urine is dark brown in color. The level of bilirubin is moderately increased due to the indirect. Provocators of hemolysis are quinine, acrichine, primaquine, sulfonamides (including antidiabetics), nitrofurans, derivatives of 8-hydroxyquinoline. (Nitroxoline, etc.), nalidixic acid (nevigramone), isoniazid (tubazid), ftyvazid, PASK, antipyrine, phenacetin, amidopyrine, acetylsalicylic acid, vicasol. These drugs probably do not exhaust the list of provocateurs of hemolytic crisis with a deficiency of G-6-FDH. Its clarification prevents the provocative role of the infection itself, which became the reason for prescribing the medicine. Since the severity of the lesion is associated with the dose of the drug, its immediate withdrawal leads to the hemolytic stagnation. Sometimes there is a temporary cessation of hemolysis, despite the continued use of the provoked medication (possibly due to the release of a large number of highly persistent young forms of red blood cells into the blood). Severe hemolytic crisis can cause acute necronemphrosis with the development of severe renal failure.

A special form of the disease is a severe hemolytic crisis in newborns, whose clinical picture corresponds to Rh-conflict.

Favism - the development of acute hemolytic syndrome in some people with deficiency of G-6-FDH in response to the use of horse beans or the ingress of pollen of this plant into the lungs. The picture of the hemolytic crisis corresponds to that described with the administration of medications, but it develops earlier - a few hours after the use of these beans, severe hemolysis appears with the development of renal insufficiency. Hemolysis, triggered by the pollen of these beans, occurs a few minutes after contact with it, it proceeds easily.

Along with hemolytic crises with G6-FDH deficiency, permanent hemolysis with slight jaundice, moderate anemia, an increase in the percentage of reticulocytes in the blood, an increase in the size of the spleen is possible. The effect of the described provoking factors, including infection, increases hemolysis. The diagnosis can be reliably established only by using methods that determine the activity of G-6-FDH in erythrocytes. In the differentiation with immune hemolysis, Coombs' reaction is particularly helpful, especially in the case of a newborn's hemopathic disease, when there is no incompatibility with the rezo-antigen. Since the deficiency of G-6-FDH is common in Transcaucasia, especially in Azerbaijan, the hemolytic crisis among ethnic representatives of this region always requires the elimination of this disease. Chronic forms of elevated hemolysis must be differentiated first of all from thalassemia, hemoglobinopathies, the distribution zone of which coincides with the deficiency of G-6-FDH.

Treatment. Abolition of the medication that caused the hemolytic crisis. Riboflavin PO. 015 g2-3 times a day inside, erectile 2 ml 2 times a day in / m. In severe hemolytic crisis, 5% sodium hydrogencarbonate solution is slowly injected to prevent renal insufficiency in the stomach (rheopolyglucin is contraindicated in renal insufficiency), furosemide (lasix) - 40-60 mpg more IV, repeatedly to enhance diuresis. With the development of anuria use plasmapheresis, if necessary, hemodialysis. Severe anemia syndrome with initial signs of anemic coma may be the basis for transfusion of red blood cells. Due to the fact that sometimes the issue of blood transfusions has to be solved before establishing the nature of the hemolytic crisis, one should use washed red blood cells (better defrosted).

Prevention. Careful collection of anamnesis. Each carrier of the defect should be provided with a list of medications that are contraindicated to it and be aware of the danger of eating horse beans. Prophylactic vaccinations to carriers of this defect should be carried out only on strict epidemiological indications.

Thalassemia is a group of hereditary hemolytic anemia characterized by severe red blood cell hypochromia with a normal or elevated serum iron level. Often the content of bilirubin in the blood is increased, moderate reticulocytosis, enlarged spleen are noted. In our country, the disease is prevalent mainly in Azerbaijan, less common in Georgia, Armenia, Tajikistan, Uzbekistan; Sporadic cases can be ubiquitous. With thalassemia, synthesis of one of the four globin chains is impaired. Inheritance of pathology from one (heterozygosity) or both parents (homozygosity), type of impaired chain determine the severity of clinical manifestations.

The pathogenesis of increased death of erythrocytes is associated with the broken structure of the cell due to an improper correlation of the globin chains in it. In addition to the shortening of the life of erythrocytes in thalassemia, the death of erythrocaryocytes of the bone marrow - the so-called ineffective erythrocytes.

The clinical picture of homozygous thalassemia is characterized by severe hypochromic anemia, sharply pronounced anisocytosis of erythrocytes, the presence of target forms (instead of normal clearing in the center, a hemoglobin spot reminding the target is determined). Early onset of hemopysis, accompanied by a sharp bone marrow hyperplasia, leads to gross violations of the facial skull (square, tower skull, saddle nose, malocclusion, dental location, etc.). The early onset of anemia causes the child's physical and mental underdevelopment. There is a slight jaundice (due to indirect bilirubin). The spleen is enlarged. Radiographically determined thickening and peculiar structure of the spongy layer of the bones of the cranial vault: "hairy" structure. Patients are prone to infectious diseases. With severe anemia, children die in the first year of life. There are also less severe forms of homozygous thalassemia, when patients survive to adulthood.

Heterozygous thalassemia can occur with moderate hypochromic anemia, a slight reticulocytosis, mild icterus, a slight increase in the spleen. However, signs of increased hemolysis with heterozygous carriage may be absent. The morphology of erythrocytes is similar to that described, although the severity of the changes is smaller.

The picture of blood in thalassemias is characterized by the morphology of erythrocytes described above, a low color index. The number of erythrocytes is normal or slightly enlarged, the leukogram, the number of platelets do not change. Diagnostic sign-normal or elevated figures of serum iron.

Diagnosis. Thalassemia may be suspected when hypochromic anemia with a pronounced anisocytosis of erythrocytes is detected, their target type (symptom is not absolutely specific), enlarged spleen, normal serum iron level. If iron-deficiency anemia is accompanied by an increase in the level of reticulocytes in the blood by the middle or end of the second week of treatment, then with thalassemia, iron intake does not change the level of reticulocytes. The form of thalassemia can be determined only by special investigation of globin chains.

Treatment of severe homozygous forms reduces to correction of anemia with the help of red blood cell transfusions in an amount that allows to keep hemoglobin at the level of 85 g / l (so-called supertransfusion), while removing excess iron by desferal. One of the early methods of therapy can be bone marrow transplantation. In severe hemolysis and a significant increase in the spleen, splenectomy is shown, but it is rarely necessary to resort to it with thalassemia.

Prevention. Since a severe homozygous form of the disease is more common in marriages of blood relatives, it is necessary to prevent such marriages.

Sickle cell anemia. In a large group of diseases caused by violations of the amino acid composition of globin, hemoglobinopathy, the most common is sickle cell anemia. The disease occurs in two forms: homozygous (severe) and heterozygous; The latter is complicated by an anemic crisis only in special conditions of oxygen starvation. By itself, the anomaly of the globin structure (replacement of the globin globin in beta chain with valine) is inherited dominantly, however clinically vivid manifestations of the disease appear only in homozygotes, ie, they are inherited recessively. Sickle-cell anemia is common in Central Africa, sporadic cases occur among people from Africa everywhere; In the CIS, foci of sickle-cell anemia are noted in Transcaucasia.

The clinical picture of the homozygous form consists of early severe hemolytic anemia, which has a normochromic or hyperchromic character. Physical and some mental retardation associated with an early developed and profound anemic syndrome, is accompanied by skeletal disorders; Children are thin with elongated limbs, the skull is peculiarly deformed, high, narrowed, often has a thickened suture of the frontal parts in the form of a crest. Thrombotic complications are characteristic. The thromboses of the vessels feeding the bones are accompanied by severe pain in the long tubular bones and joints, their swelling. Aseptic necrosis of femoral and humerus head heads is observed. Often there are thromboses of mesenteric vessels, pulmonary infarcts. Hemolytic crisis usually occurs high temperature, expressed by neutrophilic leukocytosis with rejuvenation of the formula, thrombocytosis. Massive intrasopharyngeal hemolysis in a crisis is accompanied by the release of dark urine, in which hemosiderin is determined. Often there are ulcers of the legs. At the beginning of the disease, the spleen is often enlarged, later on, due to repeated heart attacks, it wrinkles and stops palpating. The liver is also slightly enlarged.

The picture of blood: normochromic anemia, pronounced anisocytosis of erythrocytes, the presence of peculiar forms resembling a half-disk, a small number of target red blood cells. Present sickle erythrocytes are detected only with a special sample with sodium metabisulfite, which, acting as a reducing agent, causes a lack of oxygen in the erythrocytes, which leads to the crystallization of hemoglobin in them and the appearance of sickle-shaped forms. Removing the cover glass from the drug - access to oxygen - leads to the restoration of the normal form of red blood cells.

Clinical manifestations of the heterozygous form are observed only in hemolytic crises provoked by anoxic conditions (flight in an airplane without hermetic sealing of the cabin, severe pneumonia, anesthesia, etc.). The crisis is accompanied by multiple thrombotic complications. In the blood anemia, neutrophilic leukocytosis, thrombocytosis. Erythrocytes undergo the same changes as in the homozygous form, the same sample reveals sickle-shapedness. Electrophoresis of hemoglobin in homozygous form reveals the presence of only S-hemoglobin, and for heterozygous - both S- and A-fraction of hemoglobin.

Treatment. During the hemolytic crisis - hospitalization of the patient and warming, since at low temperature signs of sickle-shapedness are more expressed. Inside prescribe acetylsalicylic acid (as a disaggregant and a factor that changes the affinity of hemoglobin to oxygen) in a dose of 0.5 g 3 times a day. With severe anemia, the erythrocyte mass is poured.

In addition to sickle cell hemoglobinopathies, there are other hemoglobin anomalies, usually giving hemolytic syndrome only in the homozygous state: small jaundice, enlarged spleen, anisocytosis, and sometimes red blood cell red blood cells, reticulocytosis. Electrophoresis of hemoglobin makes it possible to clarify the nature of hemoglobinopathies. There may be heterozygosity of two pathological hemoglobins or their combination with thalassemia.

Hemolytic anemia in unstable hemoglobins. A special group of hemolytic anemia is the carriage of unstable hemoglobins; Their instability to oxidants, heating results from the replacement of amino acids in the alpha or beta chain of globin. Diseases are inherited by the dominant type, are rare, homozygous forms are absent.

The clinical picture is characterized by homolytic syndrome (jaundice, reticulocytosis, anemia, enlarged spleen). In some patients, hemolysis proceeds almost asymptomatically, in others it is expressed. Anemia is usually normochromic, anisocytosis, sometimes the body of Heinz, a small target of erythrocytes; Their basophilic puncture occurs. The last two phenomena allow us to assume the carriage of unstable hemoglobin.

Diagnosis. When various oxidizing agents, for example, brilliant crosyl blue, are exposed to erythrocytes, unstable hemoglobin undergoes denaturation and precipitates as small round inclusions. In a number of cases, the thermostability of erythrocytes is checked.

Treatment is not developed enough; With splenomegaly and expressed hemolytic syndrome splenectomy in most cases somewhat reduces the intensity of hemolysis.

Paroxysmal nocturnal hemoglobinuria (PN G, Marciafawa-Michele disease, paroxysmal nocturnal hemoglobinuria with persistent hemosiderinuria, Stryubing-Marciafawa disease) is a kind of acquired hemolytic anemia that occurs with persistent intravascular hemolysis, hemosiderinuria, suppression of granulo and thrombocytopoiesis.

The pathogenesis of the disease is associated with intravascular destruction of erythrocytes, defective in large part. Along with the pathological population of erythrocytes, a part of normal cells that have a normal life span also persists. Violations in the structure of granulocytes and platelets were detected. The disease is not hereditary, but any external factors provoking the formation of a defective cell population that is a clone, that is, the offspring of one originally altered cell, are not known. Thrombotic complications in APG are associated with intravascular hemolysis, which provokes thrombus formation. The origin of an important but far from obligatory sign of the disease - paroxysms of hemoglobinuria at night or in the morning - remains unclear. Paroxysm is not associated with the time of day, but with sleep, which during the day can also cause a crisis. There is an increased complement sensitiveness of pathological red blood cells in APG. Perhaps this is the basis for provoking the hemolytic crisis by transfusion of fresh blood, which contains factors that activate complement. Transfusion of blood stored for more than a week, hemolysis does not provoke.

Clinical picture. The disease develops slowly: there are signs of mild anemia, weakness, fatigue, palpitation during exercise, abdominal pain, often associated with thrombosis of mesenteric vessels. Skin and mucous membranes are pale yellowish, grayish in connection with anemia and deposition of hemosiderin. Characteristic features of intravascular hemolysis. The appearance of black urine is a non-permanent sign. Since APG is often accompanied by leukopenia (mainly due to granulocytopenia), chronic infectious complications are possible. Thrombocytopenia can be complicated by hemorrhagic syndrome. Prolonged release of urine hemoglobin and hemosiderin gradually leads to the development of iron deficiency - there is asthenic syndrome, dry skin, brittle nails.

The blood picture is characterized first normochromic, and then hypochromic anemia, small reticulocytosis (2-4% and more), leukopenia and thrombocytopenia. The morphology of erythrocytes has no characteristic features. In the bone marrow, hyperplasia of the red sprout is observed, but in trepanate there is a slight increase in the cellularity of the bone marrow, which, as the disease develops, may become hypoplastic. Due to the ever-ongoing intravascular hemolysis, free hemoglobin is raised in the plasma (normally less than 0.05 g / l). The level of serum iron is normal at first, then it can be significantly reduced. Along with the typical onset of the disease, when the hemolytic syndrome predominates, a picture of the aplastic syndrome may develop, which in a few years may be complicated by a haemolytic crisis with typical nocturnal hemoglobinuria. More often hemolytic crisis provokes a blood transfusion.

The diagnosis is made on the basis of signs of intravascular hemolysis (anemia, small reticulocytosis, hemosiderin in the urine). Specify the diagnosis of special studies (positive sucrose test, Hema test, negative Coombs test).

A hemolysin form of autoimmune hemolytic anemia, which proceeds with intravascular hemolysis, is similar in appearance to APG, characterized by the presence of hemolysins in the blood serum, positive breakdown of Coombs. Unlike APG, there is no leukopenia and thrombocytopenia, usually prednisolone gives a good effect. The bone marrow pattern can be distinguished from aplastic anemia of APG: at aplasia, trepanate is characterized by fat predominance, hemolysis with cellular hyperplasia, but in rare cases APG can develop a bone marrow hypoplasia, although hemosiderin is constantly found in the urine, and reticulocytosis in the blood.

Treatment in the absence of severe anemia is not carried out. Severe anemic syndrome requires transfusion of red blood cells; The best results are obtained by transfusion of washed or matured erythrocytes during 7-10 days. When hypoplasia of hematopoies, anabolic steroids are shown: nerobol 10-20 mg per day or retabolil 50 mg IM for 2-3 weeks. Apply iron preparations, but they can sometimes provoke a hemolytic crisis. To prevent the crisis, iron is prescribed in small doses against the background of treatment with anabolic steroids. In thrombosis, heparin is indicated: at the first injection, 10,000 units of ED are injected, followed by 5000-10,000 units 2-3 times a day under the skin of the abdomen (with a thin needle at a depth of 2 cm in adipose tissue) under the control of blood coagulation. Contraindications to the treatment with heparin - the recent exacerbation of peptic ulcer of the stomach or duodenum, as well as the presence of sources of bleeding.

Immune hemalitic anemia develops under the influence of antibodies on erythrocytes. The main forms of immune hemolytic anemia: 1) autoimmune, caused by the appearance in the body of antibodies against their own erythrocytes; 2) haptens caused by fixation on the erythrocytes of foreign antigens-haptens (drugs, viruses, etc.) with antibodies formed in response to the hapten's combination with the body protein; 3) isoimmune, associated with the entry into the body of newborn antibodies to the mother directed against the baby's erythrocytes (with the incompatibility of the child and the mother according to Rh-antigen and much less often in antigens of the ABO system).

Autoimmune geopolitical anemia. At the heart of the pathological process is the failure of immunolytic insensitivity to its own antigen.

The clinical picture of the disease consists of signs of anemic syndrome; The severity of the condition of patients is determined by the severity and severity of anemia. In a chronic, slowly developing process, the first sign of the disease may be a slight jaundice (due to indirect bilirubin); At the same time, anemia is detected. In other cases, the onset of the disease is characterized by violent hemolysis with rapidly increasing anemia and jaundice. Often the body temperature rises. The spleen is often enlarged. Perhaps an increase in the liver due to cholelithiasis, fatty degeneration. Usually, a functional systolic murmur is heard at the apex and at the base of the heart, often a split of the second tone is determined.

The blood picture is characterized by normochromic anemia (with acute hemolysis, the hemoglobin level may drop to catastrophic figures, the patient may fall into anemic coma). The morphology of erythrocytes does not change significantly, but sometimes their microspherocytosis is noted, which requires differentiation from hereditary microspherocytosis. In acute hemolysis, single erythrocaryocytes can be detected in the blood. The level of reticulocytes is high. White blood does not change significantly, but the hemolytic crisis may be accompanied by a short neutrophilic leukocytosis (the appointment of steroid hormones during hemolysis may be accompanied by very high neutrophilic leukemia with a shift to promyelocytes). The number of platelets is usually normal. However, there is an autoimmune cytolysis involving two germs - a platelet and erythrocyte (Ivens-Fisher syndrome). In this case, the signs of hemolytic anemia and thrombocytopenic purpura are clinically determined. Bone marrow in autoimmune hemolytic anemia is characterized by irritation of the red shoot (with a combination of hemolysis with thrombocytopenia in the bone marrow there is a high megakaryocytosis), but it is often possible to meet a high percentage of reticular cells - peculiar elements with a rough structure nucleus with a nucleolus and a wide cytoplasm containing a scant azurophilic granularity. Biochemical studies show, in addition to hyperbilirubinemia, an increase in the content of gamma globulins, which in some cases may have a monoclonal nature.

It is impossible to predict the course of the hemolytic process, the dose of the prescribed steroid hormones is often increased (the first dose is usually 60 mg per day) if there is no response to the first. The disease can manifest as a single episode of hemolysis or take a chronic course. Along with this most frequent form of autoimmune hemolytic anemia, in which erythrocytes damaged by autoantibodies are absorbed by the macrophage cells of the spleen (intracellular hemolysis), a form of the disease with intravascular hemolysis occurs. A distinctive feature in this case is the release of dark urine due to hemoglobinuria and hemosiderinuria. In severe hemolysis, thromboses are possible in the system of mesenteric vessels with the appearance of severe paroxysmal pain in the abdomen and swelling due to intestinal paresis. As a rule, small vessels and small intestine gangrene are not thrombosed, the need does not arise in the surgical intervention. Sometimes, intravascular hemolysis can occur in response to cooling (cold hemoglobinuria). This form of hemolytic anemia is associated with the action of hemolysins of blood serum on the erythrocytes of the patient.

Occasionally (mostly elderly streets), there are forms of intracellular hemolysis caused by cold agglutinins to erythrocytes. A provocateur of such hemolysis is usually a sharp cooling. The disease acquires a chronic course. Patients, as a rule, do not tolerate cold; Possible development of gangrene of fingers after a long stay in the cold. A specific feature of this process is the autoagglutination of erythrocytes during the collection of blood at room temperature, which can interfere with the determination of the level of erythrocytes and ESR. Blood can agglutinate in a smear, in a test tube. When heated agglutination disappears. In the serum of patients in the determination of protein fractions, a gradient of monoclonal immunoglobulins belonging to cold antibodies is found.

The diagnosis of autoimmune hemolytic anemia is established on the basis of common signs of hemolysis (increased bilirubin level in the blood or the appearance of hemosiderin in the urine, an increase in the percentage of reticulocytes in the blood and the detection of autoantibodies on the surface of erythrocytes with the help of Coombs test, which is positive in almost 60% of cases of autoimmune hemolysis). A higher percentage of positive findings gives the aggregate a hemagglutination test. Both samples can be delivered in specialized institutions. With a diagnostic chain, but only in exceptional cases, steroid hormone therapy can be performed that reduces or eliminates elevated hemolysis within a few days. Diagnosis and development of a specific treatment program for autoimmune hemolytic anemia is carried out under the conditions of a specialized institution. Follow-up treatment is usually performed on an outpatient basis.

Treatment. Initiate therapy usually with the appointment of 60 mg of prednisolone (for adults) per day. In severe hemolytic process with rapidly progressing anemia, the dose of prednisolone is rapidly increased to 100-200 mg per day. The ineffectiveness of steroid therapy, detected by the increase in anemia, the percentage of reticulocytes, may require splenectomy already at the very beginning of the disease. Usually the question of splenectomy has to be solved after several months of conservative treatment. According to vital indications and with ineffectiveness of splenectomy, immunosuppressants (cyclophosphamide, azathioprine, methotrexate, etc.) are prescribed. In severe anemic syndrome, per