3. Semiotics of damage to the circulatory system. Research methodology To assess the state of the cardiovascular system, an ECG study is used. The technique of taking an ECG, the lead system and the theoretical basis of the method are common to all ages. However

5. Semiotics and syndromes of damage to the cardiovascular system in children of the first year of life. Ventricular septal defect is characterized by a rough systolic murmur along the left edge of the sternum with a maximum in the IV intercostal space, at the left edge of the sternum, but usually not

LECTURE No. 13. The blood system and hematopoietic organs in children 1. Features of the blood system in children The fetus experiences a constant increase in the number of red blood cells, hemoglobin content, and the number of leukocytes. If in the first half of intrauterine development (up to 6 months) in

DISEASES OF THE HEATING SYSTEM

Lymphoid system (organs of hematopoiesis and immune system) Immunity Immunity (lat. immunitas - “liberation from something”) the body’s protection from genetically foreign organisms and substances, which include microorganisms, viruses, worms, various proteins, cells, including

Diseases of the hematopoietic organs Bee products have a pronounced effect on the processes of hematopoiesis. For example, bee venom increases the amount of hemoglobin in the blood, reduces cholesterol levels, increases the permeability of the walls of blood vessels,

Lymphoid system (organs of hematopoiesis and immune system) One of the main conditions for human existence is the body’s ability to resist the introduction of infection or other foreign substances. This function is performed by the protective system. Immunity (from lat.

Diseases of the cardiovascular system and hematopoietic organs The vascular system is a powerful branched tree that has roots, a trunk, branches, and leaves. Every cell of our body owes its life to a blood vessel - a capillary. Take everything from the body

Anemia is a condition characterized by a decrease in the amount of hemoglobin per unit volume of blood, often with a simultaneous decrease in the number of red blood cells. Anemia is considered to be a decrease in the content of hemoglobin in the blood less than 100 g/l, erythrocytes less than 4.0 × 10 12 / l and serum iron less than 14.3 µmol/l. The exceptions are iron deficiency anemia and thalassemia, in which the number of red blood cells is normal.

There are different classifications of anemia:

1. Based on the morphology of erythrocytes, anemia is divided into microcytic, normocytic and macrocytic. The main criterion for this division is the average erythrocyte volume ( SEA):
   • microcytosis - SER less than 80 fl.,
   • normocytosis - SES - 80-95 fl.,
   • macrocytosis - SER more than 95 fl.
2. Hypochromic and normochromic anemias are determined by the degree of hemoglobin saturation. The second part of the term, “chrome,” refers to the color of the red blood cells.

In accordance with these classifications there are:

• hypochromic microcytic anemia (small, pale red blood cells; low SES);
• macrocytic anemia (large red blood cells; increased SER).
• normochromic normocytic anemia (cells normal size and type, normal SEO).

According to the severity of anemia there are:

• mild degree (hemoglobin 91 - 119 g/l),
• moderate severity (hemoglobin 70 - 90 g/l),
• heavy (hemoglobin less than 70 g/l).

There is a pathogenetic classification of anemia:

1. Anemia caused by impaired hemoglobin synthesis and iron metabolism, characterized by microcytosis and hypochromia (iron deficiency anemia, anemia in chronic diseases, sideroblastic anemia, thalassemia).
2. Anemia caused by impaired DNA synthesis in conditions of deficiency of vitamin B 12 or folic acid (macrocytic anemia).
3. Normochromic normocytic anemias that have no common pathogenetic mechanism and divided depending on the reaction of the bone marrow to hypoplastic and aplastic, hemolytic and posthemorrhagic anemia.

It should be noted that there are conditions that are characterized by signs of anemia, but are not accompanied by a decrease in hemoglobin or red blood cells, but are manifested by a violation of the relationship between the number of red blood cells and plasma volume (hydremia in pregnancy, overhydration in heart failure, chronic renal failure) and a decrease in plasma volume (dehydration, peritoneal dialysis, diabetic acidosis).

In anemia, the main pathogenetic significance is hypoxia of organs and tissues with the possible subsequent development of degenerative processes. There are compensatory mechanisms aimed at reducing and eliminating the consequences of hypoxia. These include hyperfunction of the cardiovascular system, caused by the effect of under-oxidized products on the regulatory centers of the cardiovascular system. In patients, heart rate and minute volume increase, and total peripheral resistance increases.

Compensatory mechanisms also include: an increase in the physiological activity of red blood cells, a shift in the oxyhemoglobin dissociation curve and an increase in the permeability of the vascular wall to blood gases. It is also possible to increase the content and activity of iron-containing enzymes (cytochrome oxidase, peroxidase, catalase), which are potential oxygen carriers.

The clinic of anemic syndrome is characterized by general anemic complaints of general weakness, dizziness, tendency to faint, palpitations, shortness of breath, especially during physical exertion, stabbing pains in the area of ​​the heart. When hemoglobin decreases to less than 50 g/l, signs of severe heart failure appear.

During the examination, pallor of the skin, tachycardia, a slight increase in the left border of relative cardiac dullness, systolic murmur at the apex, and a “spinning top murmur” in the jugular veins are revealed. An ECG can reveal signs of left ventricular hypertrophy and a decrease in the height of the T wave.

Sideropenic syndrome

This syndrome is characterized by a decrease in iron content in the blood, which leads to a decrease in hemoglobin synthesis and its concentration in erythrocytes, as well as a decrease in the activity of iron-containing enzymes, especially α-glycerophosphate dehydrogenase.

Clinically, the syndrome is manifested by a change in taste, addiction to eating chalk, toothpaste, clay, raw cereals, raw coffee, unrefined sunflower seeds, starched linen (amylophagia), ice (pagophagia) and the smell of clay, lime, gasoline, acetone, printing ink .

Dryness and atrophy of the skin, fragility of nails and hair, hair loss are noted. The nails are flattened, sometimes have a concave (spoon-shaped) shape (koilonychia). The phenomena of angular stomatitis, atrophy of the papillae of the tongue and its redness develop, swallowing is disturbed (sideropenic dysphagia, Plummer-Vinson syndrome).

In laboratory studies, a decrease in serum iron (less than 12 μmol / l), an increase in total iron-binding capacity (more than 85 μmol / l), a decrease in the content of ferritin in the blood are revealed. IN bone marrow decrease in the number of sideroblasts.

Hemorrhagic syndrome

Hemorrhagic syndrome is a pathological condition characterized by a tendency to internal and external bleeding and the appearance of various sizes of hemorrhages. The reasons for the development of hemorrhagic syndrome are changes in the platelet, plasma and vascular components of hemostasis. Accordingly, there are three groups of diseases manifested by hemorrhagic syndrome.

First group of diseases

The first group includes diseases in which the number and functional properties of platelets are changed (thrombocytopenia, thrombocytopathy). The clinic of this group of diseases is characterized by the appearance on the skin and mucous membranes of "bruises" of various sizes and small punctate hemorrhages (petechiae). Also characteristic is the development of spontaneous bleeding - nasal, gingival, gastrointestinal, uterine, hematuria. The intensity of bleeding and the amount of blood lost are usually insignificant. This type of bleeding is typical, for example, for Werlhof's disease (thrombocytopenic purpura) and is accompanied by an increase in the duration of bleeding, impaired retraction of the blood clot, a decrease in the retention index (adhesiveness) and platelet count. Symptoms of tourniquet and pinch and cuff test are positive.

Second group of diseases

The second group includes diseases in which bleeding is caused by hereditary or acquired deficiency of procoagulants or an increased content of anticoagulants, leading to clotting disorders (hemophilia, hypo- and afibrinogenemia, dysprothrombinemia). Patients develop massive hemorrhages in soft fabrics and joints (hemarthrosis). There are no petechiae. Prolonged nosebleeds, bleeding from the gums after tooth extraction and bleeding after open injuries to the skin and soft tissue are possible. Laboratory signs - prolongation of blood clotting time, changes in autocoagulation test parameters.

Third group of diseases

The third group consists of diseases in which vascular permeability is altered (Rendu-Osler hereditary telangiectasia, Henoch-Schönlein hemorrhagic vasculitis). Rendu-Osler disease is characterized by telangiectasias on the lips and mucous membranes and can manifest as hemoptysis, intestinal bleeding, and hematuria. With Henoch-Schönlein disease, a pinpoint hemorrhagic rash appears on an inflammatory background. The rash rises slightly above the surface of the skin and gives a slight sensation of volume upon palpation. Hematuria may be observed. Standard coagulation tests were not changed.

Hemolytic syndrome

The syndrome includes diseases characterized by a decrease in the number of red blood cells and hemoglobin concentration in the blood due to increased hemolysis of red blood cells.

Increased destruction of red blood cells is due to the following factors:

• changes in the metabolism and structure of membranes, the stroma of red blood cells and the hemoglobin molecule;
• the damaging effect of chemical, physical and biological factors of hemolysis on the erythrocyte membrane;
• slowing down the movement of red blood cells in the intersinus spaces of the spleen, which contributes to their destruction by macrophages;
• increased phagocytic activity of macrophages.

Indicators of hemolysis are:

• an increase in the formation of free bilirubin and a corresponding change in pigment metabolism;
• change in osmotic resistance of erythrocytes;
• reticulocytosis.

In clinical practice, the following syndromes are distinguished, reflecting changes in the blood system. Anemic. Hemorrhagic. Hemolytic. DIC syndrome. ANEMIC SYNDROME Anemia is a condition characterized by a decrease in the hemoglobin content per unit volume of blood (often with a simultaneous decrease in the number of red blood cells), accompanying both hematological diseases themselves and many other diseases. When studying the medical history, attention is paid to the patient’s contact with toxic substances, drug intake, and symptoms of other diseases that can lead to anemia. In addition, it is necessary to assess the patient’s dietary habits and the amount of alcohol consumed. A family history of anemia should also be clarified.

Causes. Anemia can accompany various diseases of an infectious and inflammatory nature, diseases of the liver, kidneys, connective tissue, tumors, endocrine diseases. Anemia can occur acutely as a result of blood loss and hemolysis or develop gradually. The causes of microcytic anemia can be iron deficiency in the body, a violation of the inclusion of iron in erythrocytes due to changes in the synthesis of porphins (sideroblastic anemia), a defect in the synthesis of globin in thalassemia, chronic diseases, lead intoxication. Macrocytic anemia occurs when there is a deficiency of vitamin B 12 or folic acid, as well as due to toxic effect medicines.

Manifestations Anemic syndrome is accompanied primarily by clinical signs due to oxygen "starvation" of many organs. Insufficient supply of oxygen to peripheral tissues - pallor of the skin and mucous membranes; symptoms of cerebral hypoxia - dizziness, fainting. Deterioration of exercise tolerance, weakness, fatigue, shortness of breath. Compensatory changes on the part of the CCC (increased work to improve the supply of oxygen to peripheral tissues). Laboratory changes (primarily a decrease in hemoglobin levels). At a hemoglobin concentration below 50 hl, the development of heart failure is possible. It should be remembered that in the case of a gradual increase in anemia up to a decrease in hemoglobin content of less than 70-80 hl, the inclusion of compensatory mechanisms will burn to delay the appearance of clinical signs in the patient. In addition to the above manifestations, it is possible to detect lymphadenopathy, enlargement of the spleen and liver.

Compensatory changes. For anemia, manifestations from the CCC are very characteristic, associated with a compensatory reaction to an insufficient supply of oxygen to peripheral tissues (usually with a hemoglobin content of less than 100 hl), - heart rate and minute volume increase; often these changes are accompanied by the appearance of a systolic murmur at the apex of the heart. Also characteristic is a decrease in OPSS due to tissue hypoxia and a decrease in blood viscosity. One of these most important compensatory mechanisms includes a shift in the oxyhemoglobin dissociation curve, which facilitates the process of oxygen transport into tissues. Laboratory changes. For anemia, in addition to hemoglobin indicators and the number of red blood cells, it is necessary to have data on hematocrit, the number of reticulocytes, leukocytes, and platelets in the peripheral blood. Anemias are classified according to laboratory signs microcytic, macrocytic and monocytic. Determination of the color indicator of blood and MCH (this criterion is more objective) makes it possible to classify anemia into hyper-, hypo- and normochromic. By content. Reticulocyte anemia in the blood is divided into hyporegenerative and hyperregenerative.

Classification of anemia. There are several approaches to the division of anemia. From a practical point of view, it is convenient to distinguish anemia resulting from: Blood loss (acute and chronic); Insufficient formation of red blood cells; Enhanced their destruction (hemolysis); Combinations of the above factors. Insufficiency of erythropoiesis can lead to the following types of anemia. Hypochromic-microcytic anemia: with iron deficiency, disorders of its transport and utilization. Normochromic-normocytic anemia: in hypoproliferative conditions (for example, in kidney disease, endocrine pathology), hypoplasia and aplasia of the bone marrow, myelophthisis (selective violation of myelopoiesis, the process of formation of granulocytes, platelets and red blood cells in the bone marrow). Hyperchromic macrocytic anemia: with a deficiency of vitamin B 12, folic acid. Hemolysis of erythrocytes is possible with immunological disorders, intrinsic defects of erythrocytes (membranopathy, congenital enzymopathies, hemoglobinopathies).

IRON DEFICIENCY ANEMIA anemia - hypochromic(microcytic) anemia resulting from an absolute decrease in iron resources in the body. Iron deficiency in the body (with a decrease in its content in the blood plasma - sideropenia) remains common, often leading to anemia. Causes. Iron deficiency occurs as a result of three groups of causes. Insufficient intake of iron in the body. - its low content in food. - impaired absorption of iron - chronic gastrointestinal diseases, as well as gastric resection, malabsorption syndrome, celiac disease. 2. Chronic blood loss. - bleeding from the gastrointestinal tract (varicose veins of the esophagus, gastric ulcer and duodenum, hemorrhoids, ulcerative colitis, polyposis, cancer, etc.) - lung diseases (for example, a malignant lung tumor with decay). - pathology of the gynecological sphere (for example, dysfunctional uterine bleeding). 3. Increased iron consumption: during pregnancy and lactation, during growth and puberty, with chronic infections, cancer, during treatment with erythropoietin.

Clinical manifestations. Manifestations of the disease may be associated with the disease that caused the occurrence of anemic syndrome. Iron deficiency is manifested by neurological disorders in the form of paresthesia, primarily a burning sensation of the tongue. Atrophy of the mucous membrane of the tongue, esophagus, stomach, and intestines is possible. Atrophy of the mucous membrane of the larynx and pharynx can lead to dysphagia; it is considered a precancerous condition. With the gradual development of anemia, as happens in the case of prolonged blood loss, as a result of the inclusion of a number of compensatory mechanisms, complaints may be absent for a long time even with severe anemia, however, exercise tolerance in such individuals is usually reduced and returns to normal after treatment. Complaints. Typical complaints are increased fatigue and excitability, headaches, associated not so much with a decrease in hemoglobin content, but with a deficiency of iron-containing enzymes. A perversion of taste in the form of a desire to eat clay, chalk, and glue is also associated with this same factor. Physical examination. Pallor of the skin and mucous membranes, atrophic glossitis, and stomatitis are detected. Deformation of nails has rarely been observed recently. Typical changes in the cardiovascular system are also identified.

Laboratory data The following signs are found in the blood: iron deficiency anemia. A decrease in the number of red blood cells with hypochromia and, more often, microcytosis. Anisocytosis is possible. Decreased iron content in blood serum (less than 10 µmol). Increased blood levels of free transferrin and decreased saturation of transferrin with iron. Low ferritin content in blood plasma. With a slight iron deficiency, anemia can be insignificant, and often normochromic. Notes anisocytosis and poikilocytosis, later microcytosis and hypochromia appear. In some patients, leukopenia occurs, thrombocytopenia and thrombocytosis are possible. The number of reticulocytes is within normal limits and reduced. In the bone marrow, erythroid hyperplasia is possible, the severity of which does not correspond to the severity of anemia. The iron content in blood serum is also usually reduced in acute and chronic inflammation and tumor process. When examining blood after starting treatment with iron preparations, an increase in its content in the blood serum can be detected. Taking iron supplements orally should be stopped at least 24 hours before the blood test.

Diagnostics. In doubtful cases, the results of trial treatment with iron preparations orally are of diagnostic value. Adequate therapy leads to an increase in the number of reticulocytes in the blood with a peak on the 7th -10th day of treatment. A significant increase in hemoglobin levels is observed after 3-4 weeks, its normalization occurs within 2 months. Treatment. Iron supplements are prescribed. There are a significant number of iron salt preparations that can quickly eliminate iron deficiency. Iron preparations should be prescribed parenterally only in case of impaired absorption in the intestine, as well as during exacerbations peptic ulcer. The patient is recommended to eat a varied diet, primarily containing meat products.

MEGALOBLASTIC ANEMIA This is a group of diseases characterized by the megaloblastic type of hematopoiesis, when peculiar large cells, megaloblasts, appear in the bone marrow. Megaloblastic anemia is caused by impaired DNA synthesis. The main cause of megaloblastic hematopoiesis is deficiency of vitamin B 12 and folic acid. In each specific case, it is necessary to clarify the etiology of the resulting deficiency.

Normal metabolism of vitamin B 12 and folic acid Vitamin B 12 is present in food products animal origin - eggs, milk, liver, kidneys. Its absorption in the stomach requires the participation of the so-called Castle factor, a glycoprotein secreted by the parietal cells of the stomach. The minimum daily requirement for vitamin B 12 is 2.5 mcg. Since its reserves in the body are usually quite large, deficiency occurs years after the start of disruption of its intake into the body. A deficiency of B 12 creates a state of folate deficiency in the cell. At the same time, large doses of folic acid can temporarily partially correct megaloblastosis caused by B12 deficiency. Folic acid (pteroylglutamic acid) is a water-soluble vitamin found in green parts of plants, some fruits, vegetables, cereals, animal products (liver, kidneys) and is involved in in the biosynthesis of purine and pyrimidine bases. Its absorption occurs in the proximal small intestine. The daily requirement is 50 mg. Folic acid acts as a coenzyme in carbon atom transfer reactions.

Causes of vitamin B 12 and folic acid deficiency Vitamin B 12 deficiency can occur in the following cases. Limiting consumption of animal products. Impaired absorption of vitamin B 12 with the so-called pernicious anemia. In case of intestinal invasion by a wide tapeworm, which absorbs a large amount of vitamin B 12. After operations on the small intestine with the development of blind loop syndrome - in areas of the intestine through which food does not pass, the intestinal microflora absorbs a large amount of vitamin B 12. Gastrectomy. Resection of the small intestine, ileitis, sprue, pancreatic diseases. The effect of certain drugs (for example, anticonvulsants). Causes of folic acid deficiency. Errors in diet. Insufficient consumption of plant foods, especially with alcohol abuse and in children. Impaired absorption of folic acid due to damage to the small intestine (for example, tropical sprue). An increased need for vitamin B 12 and folic acid occurs during pregnancy, hyperthyroidism, and tumor diseases.

Clinical manifestations. In the clinical picture of the disease, manifestations of both vitamin B12 deficiency itself and megaloblastic anemia are observed. Manifestations of vitamin B 12 deficiency. With vitamin B 12 deficiency, characteristic Gunter's glossitis, weight loss, neurological disorders, and a positive reaction to the administration of vitamin B 12 are noted with an initially low level in the blood serum. Neurological disorders caused by demyelination are very characteristic - the so-called funicular myelosis, manifested primarily by symmetrical paresthesias in the legs and fingers, disturbances in vibration sensitivity and proprioception, and progressive spastic ataxia. He also observes increased irritability, drowsiness, changes in taste, smell, and vision.

Manifestations of megaloblastic anemia. The main clinical manifestations of megaloblastic anemia of any origin are the same and depend on the degree of its severity. The development of anemia usually occurs rather slowly, so an asymptomatic course is possible until the hematocrit decreases significantly. At this stage, the clinical symptoms of anemia are nonspecific - weakness, fatigue, palpitations, shortness of breath during exercise, then the damage to the heart muscle increases with the appearance of various changes on the ECG, expansion of the heart chambers up to the development of congestive heart failure. Patients are pale, subicteric, and have a puffy face. Sometimes an increase in body temperature to subfebrile levels is observed. Gastric secretion in most patients with vitamin B12 deficiency and megaloblastic anemia is sharply reduced. Gastroscopy reveals atrophy of the mucous membrane, confirmed histologically.

Laboratory and instrumental research methods. The most common changes in peripheral blood during megaloblastic anemia include the following: Macrocytosis, the main symptom of megaloblastic anemia, may precede the development of anemia itself and other symptoms of vitamin deficiency. Macrocytosis in peripheral blood is assessed by color index or, more reliably, by MCV. Poikilocytosis and anisocytosis are also detected. In the blood smear, Jolly bodies are found - the remains of normoblast nuclei detected in erythrocytes (red blood cells normally do not contain nuclei). Cabot rings are morphological formations in red blood cells in the shape of a ring, figure eight or treble clef, which are probably remnants of the nuclear envelope. Decreased hemoglobin content. Of particular importance is the increase in reticulocytes, which occurs on days 3-5 of treatment and reaches a maximum on day 10.

Bone marrow research. Megaloblasts are found in the bone marrow. Myeloid cells are usually enlarged: giant metamyelocytes and erythroid hyperplasia are detected. An important diagnostic test is the reaction to the administration of vitamin B 12; with repeated sternal puncture after 8-12 hours, a transition from megaloblastic to erythroblastic hematopoiesis is noted. Determination of the content of vitamin B 12 in the blood. In addition to the study of bone marrow hematopoiesis, for the diagnosis of B 12 and folic acid deficiency conditions, determination of the concentration of these substances in the blood is currently used. Low levels of vitamin in the blood are observed with a deficiency of vitamin B 12 in food, folic acid deficiency, pregnancy, taking oral contraceptives, very large doses of vitamin C, transcobalamin deficiency, multiple myeloma. The reasons for a false increase in vitamin B 12 in the blood are myeloproliferative diseases, hepatocarcinoma and other liver diseases, autoimmune diseases, lymphoma.

Pernicious anemia (pernicious anemia) is the classic and most striking example of megaloblastic B 12 deficiency anemia. This is a disease that develops as a result of insufficient absorption of vitamin B 12, caused by impaired secretion of the internal factor of Castle and manifested by hyperchromic anemia, signs of damage to the gastrointestinal tract and nervous system. Disturbances in the synthesis of Castle factor are associated with autoimmune damage to the gastric mucosa due to hereditary predisposition, atrophy of the gastric mucosa with achlorhydria. The following factors indicate the autoimmune nature of the disease: Detection of antibodies to parietal cells of the gastric mucosa in the blood serum of 90% of patients and only in 10% of the control group suffering from atrophic gastritis without anemia. Identification of antibodies that bind to internal factor or the “intrinsic factor-vitamin B 12” complex. The combination of pernicious anemia with thyrotoxicosis, hypothyroidism and Hoshimoto's goiter, in the pathogenesis of which an autoimmune mechanism is involved, and autoantibodies to thyroglobulin and rheumatoid factor are often simultaneously detected. Reversal of symptoms of the disease under the influence of glucocorticoids.

Enzymopathy of erythrocytes Hereditary deficiency of erythrocyte enzymes manifests itself most often when the body is exposed to certain toxins and medicinal substances in the form of acute hemolysis, less often chronic. Among them, the most common is deficiency of the G-6-PD enzyme, which is involved in maintaining the normal intracellular content of reduced nucleotides. The severity of the disease depends on the severity of the deficiency. A slight deficiency is manifested by acute hemolysis when taking drugs that exhibit oxidative properties, which was first described during treatment with prihamin. Later, the effects of other antimalarial drugs, sulfa drugs, and nitrofuran derivatives became known. Liver and kidney failure favors acute hemolysis due to G-6-PD deficiency. Severe enzyme deficiency is characterized by the development of neonatal jaundice, as well as spontaneous chronic hemolysis. A simple indicative diagnostic test is the detection of Heinz-Ehrlich bodies in erythrocytes. Spontaneously or after incubation in the presence of phenylhydrazine, a significant proportion of G-6-PD-deficient erythrocytes show inclusions, which are precipitates of hemoglobin derivatives.

Hematology (from the Greek blood and study) is a section of internal medicine that studies the etiology, pathomorphology, pathogenesis, clinical picture and treatment of diseases of the blood system. Hematology studies embryogenesis, morphogenesis, morphology and physiology of cellular elements of blood and hematopoietic organs, properties of plasma and serum, symptomatic changes in hematopoiesis in non-hematological diseases and exposure to ionizing radiation. In 1939, G. F. Lang included in the concept of the blood system: blood, hematopoietic organs, hematopoiesis, and the neurohumoral apparatus for regulating hematopoiesis and hemodestruction.

Often the main complaints of patients are general weakness, fatigue, drowsiness, headaches, dizziness. An increase in temperature can occur with hemolytic anemia due to the pyrogenic effect of the breakdown products of erythrocytes, as well as with leukemia, especially with leukemic forms. Septic complications are often associated in the form of necrotic tonsillitis, gingivitis, stomatitis. Lymphogranulomatosis is characterized by undulating undulating fever, with gradual rises and then drops in temperature over 8-15 days. Typical for blood diseases is hemorrhagic syndrome, which is characterized by a tendency to nasal, gastrointestinal, renal, uterine bleeding, as well as the appearance of hemorrhagic rashes on the skin in the form of punctate elements - petechiae and bruises (ecchymosis). Itchy skin may precede the appearance of detailed clinical symptoms, which is especially typical for lymphogranulomatosis, hematosarcomas, and erythremia. Pain in the bones, mainly in the flat bones, is typical for acute leukemia in children. Bone pain and pathological fractures are characteristic of myeloma.

A number of symptoms are associated with an enlarged liver and spleen. I am worried about pain in the right or left hypochondrium. The pain can be dull or severe sharp pains occur when the spleen is infarcted, when it ruptures, or when perisplenitis occurs. With anemia, in particular iron deficiency and chlorosis, taste distortions occur: patients eat chalk, clay, earth (geophagy). There may be disturbances in the sense of smell: patients like to inhale vapors of gasoline, ether and other odorous substances. The patient may notice an increase in lymph nodes and consult a doctor with this complaint. A burning sensation at the tip of the tongue and along its edges occurs periodically and often reaches such a degree that it becomes difficult to eat spicy and hot food. These sensations are associated with inflammatory changes in the mucous membrane of the tongue (Gunter's glossitis), which is a typical sign of B-12 folate deficiency anemia. It is necessary to find out from the patient’s life history whether the patient has encountered occupational hazards: working with benzene, mercury salts, lead, phosphorus, which can cause agranulocytosis; Determine the presence of radiation exposure (acute leukemia, chronic myeloid leukemia). Some blood diseases such as hemophilia and hemolytic anemia can be inherited. Bleeding and chronic diseases of internal organs play a role in the development of anemia. Taking medications, in particular chloramphenicol, pyramidone and butadione, can contribute to the development of agranulocytosis.

Thrombocytopenia (thrombopenia) - a decrease in the number blood platelets- characteristic of thrombopenic purpura, often occurs in severe forms of anemia and leukemia. It would be a mistake to think that based on the morphological characteristics of the blood alone one can always make the correct diagnosis and prognosis of the disease; this is possible only in isolated cases. In most cases, a hemogram acquires diagnostic and prognostic value only if all clinical signs are simultaneously assessed; Of particular value is taking into account the dynamics of changes in the blood during the course of the disease. For a correct assessment of the state of hematopoiesis, especially great importance has a study of intravital bone marrow punctures (myelogram), lymph nodes and spleen.

Neutropenia (decreased number of neutrophils) is observed in lymphatic children, with tuberculosis, anaphylactic shock, severe forms of influenza and typhoid fever; neutropenia with leukopenia - in severe forms various infections and sepsis, as well as with long-term administration of sulfonamide drugs, embiquin, etc. Neutropenia reaches sharp degrees with agranulocytosis and aleukia. Eosinophilia can be pronounced (although not always) with exudative diathesis, bronchial asthma, after injections of foreign serums, with scarlet fever, trichinosis, echinococcus and some other forms of helminthiasis, with lymphogranulomatosis and with the so-called “eosinophilic pulmonary infiltrates”. An increase in the number of eosinophils during acute infections is a prognostic sign in most cases. Eosinopenia (decrease in the number of eosinophils) is observed in acute infectious diseases (with the exception of scarlet fever), especially in typhoid fever, measles, sepsis, pneumonia, etc. Complete disappearance of eosinophils (aneosinophilia) is often observed in malaria, leishmaniasis; in other infections, this is an unfavorable prognostic sign.

Lymphocytosis is observed with lymphatic and exudative diathesis, with rickets (often with monocytosis), rubella and some other infections. Lymphopenia occurs with most febrile infectious diseases, with lymphogranulomatosis, miliary tuberculosis and some myelosis. Monocytosis is most pronounced in monocytic tonsillitis, often with measles, scarlet fever, malaria and other infections. Monocytopenia occurs in severe septic and infectious diseases, malignant forms anemia and leukemia. Thrombocytosis often occurs with pneumonia, rheumatism and other infectious diseases.

Anemia is a polyetiological disease characterized by changes in external signs (pallor of the skin, mucous membranes, sclera, often masked by jaundice), the appearance of disorders of the muscular system (weakness, decreased tissue turgor), abnormalities in the central nervous system (lethargy, apathy, mild excitability) , functional disorders cardiovascular system (tachycardia, expansion of boundaries, the appearance of systolic murmur in Botkin’s point and the apex of the heart), the development of hepato- and splenomegaly, changes in the morphology of red blood cells (decrease in volume, change in shape, osmotic resistance), changes in the content of other cellular forms (leukocytes, platelets) bone marrow punctate, electrolyte metabolism and iron and magnesium content in blood serum.

The classification is as follows.

1. Deficiency anemia: iron deficiency, vitamin deficiency, protein deficiency.

2. Hypo- and aplastic anemia: congenital Fanko-ni anemia, Dabionda-Biekfen anemia, acquired anemia.

3. Hemolytic anemia: spherocytic, sickle cell, autoimmune.

By severity:

1) mild anemia: hemoglobin within 90 – 110 g/l, the number of red blood cells decreases to 3 minutes;

2) anemia moderate severity: hemoglobin 70–80 g/l, red blood cells up to 2.5 min;

3) severe anemia: hemoglobin below 70 g/l, red blood cells below 2.5 min. By functional status erythropoiesis:

a) regenerative anemia: retiulocytes more than 50%;

b) hypo- and aregenerative anemias: low retilunocytosis, inadequate severity of anemia. According to the course: acute phase, subacute and chronic course.

Iron-deficiency anemia

Iron deficiency anemia is a disease that is caused by iron deficiency in the blood serum, bone marrow and depot, which leads to the development of trophic disorders in tissues. The development of anemia is preceded by latent tissue iron deficiency. It is more common in women than in men, in 14% of women of childbearing age living in the middle zone.

Etiology: The causes of iron deficiency anemia are chronic blood loss, insufficient initial iron levels, which manifest themselves during puberty; impaired absorption and intake of iron from food. Most often several are combined unfavorable factors. Agistral and enthologenic anemias are often accompanied by a deficiency of not only iron, but also vitamin B12, folic acid, and proteins.

Classification:

1) chronic posthemorrhagic;

2) conditioned hemoglobinuria and hemosiderinemia;

3) iron deficiency in donors (withdrawal of 400–500 ml of blood is accompanied by a loss of 200–250 mg of iron.).

Clinic. History of insufficient, incorrect, one-sided nutrition, frequent illnesses. Dryness, roughness of the skin, brittle hair, pallor of the mucous membranes, atrophy of the papillae of the tongue; functional changes in the gastrointestinal tract, leading to spasms of the esophagus, accelerated intestinal motility, spleno- and hepatomegaly.

Changes in the morphology of erythrocytes and biochemical parameters of the blood serum, anisocytosis, poikilocytosis, a decrease in the osmotic ability of erythrocytes, a decrease in the concentration of serum iron, an increase in the copper content in the blood serum.

In the mechanism of development of clinical manifestations of iron deficiency anemia, tissue hypoxia and a decrease in the activity of most enzymes are of paramount importance.

Patients experience severe weakness, darkening before the eyes when changing body position, headaches, dizziness, fainting, shortness of breath, palpitations during minor physical exertion, increased tooth decay, and smoothness of the papillae of the tongue. IN severe cases- disturbance, discomfort when swallowing (Bechterew's symptoms) of dry and solid food, crimson coloration of the tongue, atrophic changes in the pharynx and esophagus, spastic narrowing of the upper part of the esophagus, fragility, formation of longitudinal or transverse striations along the nail plate, koilonychia. A distortion of the sense of taste (addiction to honey, tooth powder, chalk, dry cereal, coal, lime, ice, the smell of gasoline, kerosene) indicates a violation of peripheral taste sensitivity. Patients may complain of muscle weakness, urinary urgency, and enuresis. Hyporegeneration of blood cells is caused by a decrease in the proliferative capacity of the bone marrow and ineffective hematopoiesis. Differential diagnosis carried out with thalassemia, posthemorrhagic and infectious anemia.

Treatment

The principles of treatment are as follows.

1. Active mode.

2. Balanced diet.

3. Iron supplements in combination with ascorbic acid and copper.

4. Aerotherapy, massage, gymnastics.

5. Food enzymes.

6. Blood transfusion when the hemoglobin content is less than 60 g/l, iron supplements are prescribed orally between meals, since better absorption occurs during this period. In case of iron intolerance (decreased appetite, nausea, vomiting, epigastric pain, dyspepsia, allergic dermatoses), iron preparations are administered parenterally to avoid damage to the gastrointestinal mucosa. Iron preparations include hemostimulin, ferroplex, sorbifer durulez, ferrum lek. Prevention of anemia should be carried out for donors, women with heavy menstruation, frequent pregnancies, girls during puberty, and with frequently recurring bleeding.

Vitamin deficiency anemia

Pernicious anemia (Addison-Birmer disease) is caused by a deficiency of vitamin B12, manifested by damage to the hematopoietic, digestive and nervous systems. Occurs more often in old age, with equal frequency in men and women.

Etiology. Vitamin deficiency is rarely exogenous, more often endogenous, associated with their increased consumption (helminthiases) and impaired absorption of various natures (stomach disease, malabsorption syndrome). Malabsorption of vitamin B 12 is most often caused by atrophy of the gastric mucosa and the absence or decrease in the secretion of internal factor, hydrochloric acid, and pepsin. There is a hereditary predisposition associated with impaired secretion of the internal factor; violation of immune mechanisms (detection of antibodies to own cells). Pernicious anemia may occur after gastrectomy, resection.

Clinic

In patients with pernicious anemia, the skin acquires a lemon-yellow tint, and spotted-brown pigmentation may form. Patients complain of weight loss caused by anorexia, and possibly an increase in body temperature. In half of the patients, symptoms of glossitis appear, sometimes the mucous membranes of the cheeks, gums, pharynx, esophagus are affected, diarrhea develops, the liver is enlarged, the spleen is often enlarged - hepatosplenomegaly; shortness of breath, palpitations, extrasystole, weakness, dizziness, tinnitus. Characteristic of pernicious anemia is rheumatic syndrome, which is caused by damage white matter spinal cord. In severe cases, signs of damage to the posterior columns of the spinal cord appear, an uncertain gait, impaired coordination of movements, ataxia, hyperreflexia, and tone of the feet. Rare but dangerous symptoms are mental disorders, maximum outbursts, and paranoid states.

Differential diagnosis performed with familial megaloblastic anemia.

Survey plan.

2. Examination of stool occult blood.

3. Blood test for reticulocytes, platelets.

4. Determination of iron content in the blood (if iron deficiency anemia is suspected).

5. Fibrogastroduodenoscopy.

6. Colonoscopy (if irrigoscopy is impossible).

7. X-ray examination lungs.

8. Sternal puncture, myelogram study.

9. Biochemical research blood (total protein and protein fractions, bilirubin, transaminases).

10. Study of the secretory function of the stomach.

Principles of treatment.

1. Diet therapy.

2. Appointment of B 12 and folic acid.

3. Enzyme therapy.

4. Anabolic hormones and insulin.

5. Stimulating therapy.

The best results are obtained by treatment with vitamin B 12 (cyanocobolamine). In severe cases, intravenous administration of the drug at 100–200 mcg for a week. The course dose is 1500–3000 mcg. In severe cases and in the presence of antibodies, the appointment of corticosteroids is indicated.

Hypo- and aplastic anemia

A group of diseases of the blood system, the basis of which is a decrease in the production of bone marrow cells, most often of three cell lines: erythrocyte-, leuko- and thrombocytopoiesis.

Diagnostic criteria. Congenital Fanconi anemia: in the analysis there is a gradual increase in pallor of the skin and mucous membranes, weakness, lethargy, combined with a lag in physical development, strabismus, hyperreflexia.

IN early childhood melanin brown pigmentation of the skin appears, abnormalities of the bones, forearms, thumbs, spinal deformity, dwarfism, combined with malformations of the heart, kidneys (heart, renal failure), sensory organs, central nervous system (microcephaly); changes in the blood: drop in hemoglobin, pancytopenia, decrease in reticulocytes, deficiency of glucose-6-phosphate dehydrogenase, decreased activity alkaline phosphatase, polysaccharides.

Congenital Estren–Dameshek anemia. In the anamnesis: manifests itself on early stage development of children with the gradual development of pallor, irritability, apathy; peculiar appearance: blond hair, snub nose, wide-set eyes, thickening upper lip with a bright red border; slowing down the rate of ossification in the wrists; spleno- and hepatomegaly, changes in the blood are expressed; increasing iron deficiency anemia; in bone marrow puncture: pallor of the brain due to the development of hypoplastic erythropoiesis.

Acquired hypo- and aplastic anemia. History: develops after viral infections, with a predisposition to allergic reactions for medications and toxins. Characterized by rapid development of clinical symptoms; temperature reaction (low-grade fever), pallor, skin rash, exanthema on the mucous membranes, stomatitis and sore throat, bloody stools, weakness, anorexia, shortness of breath, possible cerebral hemorrhages, adrenal glands:

1) changes in the blood: pancytopenia, a sharp decrease in hemoglobin, hyperchromia and macrocytosis of erythrocytes, iron content in the blood serum is normal or increased;

2) in bone marrow aspirate: fatty degeneration, poverty of formed elements, absence of young forms of megalocaryocytes.

Survey plan.

1. General analysis blood, urine.

3. Sterile puncture with myelography examination.

4. Fibrogastroduodenoscopy, colonoscopy, ultrasound examination of the liver, pancreas, kidneys (to exclude neoplasms).

Differential diagnosis. Inhibition of hematopoiesis can occur with osteosclerosis and osteomyelofibrosis. It is also necessary to differentiate aplastic (hypoplastic) anemia from acute leukemia and Werlhof's disease.

Principles of treatment.

1. Transfusion of red blood cells in acute forms.

2. Plasmaphoresis with the introduction of fresh frozen plasma, albumin or rheopolyglucin.

3. Impact on the vascular wall (dicinone, serotonin, rutin, ascorbic acid - ascorutin).

4. Glucocorticoids along with massive doses of antibiotics; B vitamins, folic acid.

5. Aminocaproic acid, anabolic hormones (reta-bolin).

6. Splenectomy.

Hemolytic anemia

These are anemias that develop due to the destruction of red blood cells.

Etiology– a group of acquired and hereditary diseases characterized by intracellular or intravascular destruction of red blood cells. Autoimmune hemolytic anemia is associated with the formation of antibodies to the red blood cells' own antibodies.

Diagnostic criteria: hereditary microspherocytic anemia (Minkowski-Shoffir disease):

1) in the anamnesis: the first symptoms are detected at any age, begin as a result of existing birth defect lipoid structures of the erythrocyte membrane, therefore identifying relatives suffering from anemia is important;

2) pallor with a lemon-yellow tint, congenital stegein (tower skull, wide bridge of the nose, high palate, lethargy, weakness, decreased appetite up to anorexia, dizziness);

3) changes in the cardiovascular system, palpitations, shortness of breath, systolic murmur;

4) changes in the gastrointestinal tract: abdominal pain, colic and significant enlargement and hardening of the liver and spleen;

5) ulcers on the legs - changes in the blood: a decrease in the number of red blood cells to 2.5 minutes, hemoglobin to 70 g/l, an increase in retinulocytes to 30–50%, a decrease in the minimum osmotic resistance of red blood cells with an increased maximum, an increase in the level indirect bilirubin, urobillinogen in urine, stercobillin in the skin;

6) in bone marrow punctate – inhibition of the erythroid germ.

Sickle cell anemia

Anemia develops in early age. Family history and detection of abnormal hemoglobin in relatives are important:

1) pallor or yellowness of the skin, mucous membranes, sclera, asthenia of the body; characteristic appearance: short torso, long thin limbs, narrow shoulders and hips, tower skull, large abdomen, ulcers on the extremities, hepatosplenomegaly, expansion of the borders of the heart, arrhythmia, systolic murmur;

2) changes in the blood: normochromic anemia 2.5–3 min, hemoglobin S or its combination with hemoglobin F, anisocytosis, poikilocytosis, target-like erythrocytes, deficiency of the enzyme glucose-6-phosphate dehydrogenase.

Autoimmune anemia:

1) history: anemia develops acutely or gradually after viral, bacterial infections, against the background of rheumatism, cirrhosis of the liver, lymphagranulomatosis, etc.;

2) pallor of the skin and mucous membranes, jaundice in 75% of cases, elevated body temperature, weakness, drowsiness, excitability, headaches, abdominal pain, back pain, splenomegaly, hematuria;

3) changes in the blood: decreased levels of hemoglobin, red blood cells, reticulocytes, bilirubinemia, increased serum iron concentration; positive Coombs test (detection of antibodies to red blood cells);

4) in urine: hemoglobinuria;

5) in bone marrow punctate: irritation of the erythrocyte process.

Survey plan.

1. General analysis of blood, urine, feces.

2. Blood test for reticulocytes, platelets.

3. Urine analysis for urobilin and bilirubin.

4. Biochemical analysis for urea, creatinine, transaminases (AIT, AST), bilirubin, total protein and protein fractions.

5. Study of osmotic resistance of erythrocytes.

6. Fibrogastroduodenoscopy.

7. Ultrasonography liver, pancreas and gall bladder.

8. Coolibs reaction (if autoimmune anemia is suspected).

Differential diagnosis. It is carried out with acute leukemia, Werlhof's disease, sepsis, and other hemolytic anemias in which spherocytosis is not detected and the osmotic resistance of erythrocytes increases (thalassemia, etc.).

Principles of treatment.

2. Transfusion of red blood cells, ear blood.

3. Corticosteroids.

4. Glucose 5% with insulin and vitamins B, B2, B12, B6, C.

5. If there is no effect - immunosuppressants, splenectomy, laser irradiation.

Hemorrhagic diathesis is a group of diseases characterized by impaired hemostasis (vascular, platelet or plasma) and manifested by an increased tendency to bleeding and hemorrhage.

Etiology

The heredity of hemorrhagic conditions is determined by abnormalities of megakaryocytes and platelets, defects in plasma coagulation factors, and inferiority of cervical blood vessels.

Acquired hemorrhagic diathesis is caused by disseminated intravascular coagulation syndrome, toxic-infectious conditions, liver diseases, and the effects of medications.

Classification

Diets differentiate.

I. A disease caused by impaired vascular hemostasis (vasopathy).

1. Shenain-Henoch disease (simple, rheumatoid, abdurate and fulminant purpura):

1) simple form;

2) chronic form.

2. Hereditary familial simple purpura (Davis).

3. Anular telangiectatic purpura of Mabocchi.

4. Necrotizing Sheldon's purpura.

5. Hyperglobulinemic Waldenström's purpura.

6. Hereditary hemorrhagic telangiectasia.

7. Louis-Barr syndrome (capillary telangiectasia of the conjunctiva with ataxia and chronic pneumonia).

8. Kasabach-Merritt syndrome.

9. Scurvy and Mimer-Barny disease.

II. Diseases caused by a violation of the platelet mechanism of hemostasis (thrombocytopathy, thrombocytopenia):

1) hemorrhagic thrombocytopathy, Werlhof's disease:

a) acute form;

b) chronic form (continuous and recurrent);

2) amegakaryocytic thrombocytopenic purpura (Landolt);

3) autoimmune thrombocytopenia of various origins;

4) thrombocytopenic hymphragic purpura with acquired autoimmune hemolytic anemia (Feemer-Evans syndrome);

5) thrombocytopenic purpura with a chronic purulent shade and exudative diathesis (Ondrich syndrome);

6) thrombotic thrombocytopenic purpura of Moshkovich;

7) thrombocytopenia in geangiomas (Kasabach-Merritt syndrome);

8) hereditary properties of thrombopathy:

a) Glanuman thrombasthenia;

b) von Willibrand thrombopathy;

9) thrombocytopathy in combination with a violation of coagulation factors.

III. Diseases caused by disorders of blood clotting factors (coagulopathy):

1) hemophilia A (factor VIII deficiency):

a) hereditary;

b) family;

c) sporadic;

2) hemophilia B (factor IX deficiency):

a) hereditary;

b) family;

c) sporadic;

3) hemophilia C (factor XI deficiency);

4) pseudohemophilia due to hypoprothrombinemia:

a) idiopathic hypoprothrombinemia;

b) secondary hypoprothrombinemia (hemorrhagic disease of newborns, impaired absorption of vitamin K, liver disease, poisoning with chloroform, phosphorus, arsenic);

5) Ouren's pseudohemophelia:

a) congenital form;

b) acquired form;

6) pseudohemophelia due to deficiency of factor VII:

a) congenital form;

b) acquired form;

7) pseudohemophelia due to a lack of fibrinogen (afibrinogenemia):

a) congenital form;

b) acquired form (DIC syndrome);

8) pseudohemophelia due to lack of factor X;

9) pseudohemophelia due to lack of fabrinase;

10) pseudohemophelia due to excess anticoagulants:

a) idiopathic;

b) immunoallergic;

c) acquired forms.

Hemorrhagic vasculitis(Shenain-Henoch disease, capillary toxicosis, anaphylactic purpura) is an infectious-toxin-allergic disease based on generalized hyperegic inflammation of blood vessels.

Etiology

The cause of the acute inflammatory process of small joints of the skin, joints of the digestive tract and kidneys is not fully understood.

Degree of activity – I, II, III.

Course: acute, subacute, chronic, recurrent.

Outcome: recovery, transition to a chronic form, outcome to chronic nephritis (A. S. Kalinichenko, 1970).

Diagnostic criteria

Clinical:

1) hemorrhagic skin syndrome: the rash is usually located symmetrically, characterized by stages of the rash, localized on the extensor surfaces of the limbs, around the ankle and knee joints, in the area of ​​the feet, less often the thighs; rashes are usually polymorphic: hemorrhagic papules, erythrimatous papules, spots; at the beginning of the disease, the rashes are urtic in nature, later they become hemorrhagic, up to necrosis, and relapses are typical;

2) articular syndrome: joint lesions are of a migratory, polyatric nature with a predominant localization in the shin, ankle, elbow, and wrist joints, and joint damage is rarely symmetrical;

3) abdominal syndrome: cramping abdominal pain of varying intensity; pain may be accompanied by intestinal and kidney bleeding.

Laboratory research: hematological changes: leukocytosis, neutrophilia, eosinophilia, accelerated ESR, platelet count is sometimes slightly reduced; blood clot retraction, bleeding duration and blood clotting time are not impaired; hypercoagulation is often detected; urine test: in acute period diseases often reveal morning proteinuria, hematuria; There may be an admixture of blood in the stool with abdominal syndrome.

Examination plan:

1) general analysis of blood, urine, feces;

2) study of platelet clotting time;

3) determination of coagulogram;

4) examination of feces for occult blood (Gregersen reaction).

Differential diagnosis

It is carried out with thrombocytopathies, thrombocytopenias, coagulopathy, toxic drug vasculitis, allergic and infectious diseases.

Principles of treatment

1. Hospitalization and bed rest for at least three weeks.

2. Diet excluding cocoa, coffee, citrus fruits, strawberries, etc.

3. Heparin therapy.

4. A nicotinic acid in combination with heparin.

5. Prednisolone.

6. Plasmapheresis (for chronic vasculitis).

Thrombocytopathies are a quantitative and qualitative deficiency of the platelet component of hemostasis, clinically manifested by hemorrhagic syndromes.

Etiology

Depending on the genesis, two groups are distinguished:

1) thrombocytopenia – a decrease in the number of platelets (Werlhoff, Wilbrand-Jurgens, Frank, Kasabach-Merritt disease);

2) thrombocytopathies - a violation of the properties of platelets. In the vast majority of cases, thrombocytopenia is observed, which is based on an immunoallergic conflict.

Classification

By type: primary (idiopathic) and secondary (symptomatic) thrombocytopenia.

Nosological forms: isoimmune, transimmune, heteroimmune, autoimmune.

Werlhof's disease

Classification of idiopathic thrombocytopenic purpura (Werlhof's disease)

Course: acute (up to 6 months); chronic: with rare relapses, with partial relapses, continuously relapsing.

Clinical picture of purpura: dry purpura (skin syndrome); wet purpura (skin syndrome and bleeding).

Immunological tests: positive, negative.

Period: exacerbation, clinical remission, clinical-gamotological remission.

Complications: uterine, gastric, intestinal bleeding, posthemorrhagic encephalopathy, etc.

Diagnostic criteria

Clinical: pallor of the skin and immune membranes:

1) hyperplastic syndrome: enlarged spleen, less often – liver;

2) hemorrhagic syndrome: hemorrhages in the skin, mucous membranes (asymmetrically located, different shapes and sizes from petechiae to ectomosis, bleeding from various organs (nasal, uterine, intestinal, etc.), positive endothemial tests (symptoms of tourniquet, pinch).

Laboratory criteria:

1) in a general blood test, a decrease in the number of platelets, changes in the morphology of the plates and their functional properties (adhesion, aggregation); impaired blood clot retraction; increased duration of bleeding, slower blood clotting; decrease in the number of red blood cells and raticunocytes during bleeding;

2) change in the myelogram: hyperplasia of the megakaryocyte lineage with impaired functional activity of megaparyocytes;

3) immunological: the presence of antiplatelet antibodies. Survey plan.

1. General analysis of blood, urine, feces.

2. Blood clotting time, bleeding duration, platelet count.

3. Coagulogram.

4. Feces for occult blood (Gregersen reaction).

5. Examination of bone marrow puncture (myelogram).

6. Immunological studies for the presence of antiplatelet antibodies.

Differential diagnosis

It is necessary to distinguish idiopathic thrombocytopenic purpura from allergic anemia, primary liver diseases in combination with splenomegaly, systemic lupus erythematosus, and genetically determined thrombocytopenia.

Treatment

Principles of treatment of thrombocytopenia:

1) prevention of bruises and injuries;

2) antibiotics for bacterial infections;

3) transfusion of plasma and large doses of β-globulin;

4) corticosteroids;

5) splenectomy;

6) immunosuppressants (azothiopril, vancristine). Principles of treatment of thrombocytopathies:

1) E-aminocaproic acid, synthetic contraceptives (bisecurin, microfollin), magnesium sulfate 25% intramuscularly, magnesium thiosulfate orally;

2) locally, subcutaneously or intramuscularly, adrenochrome monosemicarbazone (adroxon, chromadrone, adrenoxyl), dicyone;

3) intravenous administration of platelet mass.

Coagulopathies are disorders of hemostasis, which are based on a deficiency of certain plasma coagulation factors.

Etiology

Hereditary coagulopathies (they are correctly called hemophilia) are caused by a genetically determined decrease or change in plasma components of hemostasis. Acquired coagulopathies occur in infectious diseases, liver and kidney diseases, severe enteropathies, rheumatoid arthritis, etc.

Classification

Classifications of hereditary coagulopathies.

1. Hemophilia: A-deficiency of factor VIII (synthyhemophilic globulin); B-deficiency of factor IX (Christmas); C-deficiency of factor XI (precursor of plasma thromboplastin); D-deficiency XII (Hagemani).

2. Parahemophilia: deficiency of factor V (proaccelerin); deficiency of factor VII (proconvertin); deficiency of factor II (pro-trolobin); factor X deficiency (Stewart-Prower).

3. Impaired fibrin formation, deficiency of factor I (fibrinogen). Forms of flow: light, heavy, hidden.

Diagnostics

Clinical diagnostic criteria are pallor of the skin and immune membranes; hemorrhagic syndrome: hamarthrosis, hemorrhage into soft tissues due to trauma to the skin and mucous membranes (extensive hematomas); hematuria; internal hemorrhages.

Laboratory diagnostic criteria - hematological: anemic syndrome (decrease in the number of red blood cells and hemoglobin, hypochromia, reticulocytosis with bleeding), hypocoagulation syndrome of blood clotting disorders (according to Lee-White more than 10 minutes), increased recalcification time (more than 250 s), increased plasma tolerance to heparin (more than 180 s), decrease in plasma factors.

Survey plan.

1. General analysis of blood and urine.

2. Determination of blood clotting time and platelet count.

3. Coagulogram, determination of antihemophilic globulin (AGG).

4. X-ray of the affected joints.

Differential diagnosis

Carry out with thrombocytopathies, hemorrhagic vasculitis, hemarthrosis with rheumatism.

Treatment

The principles of treatment are as follows:

1) increase in the blood level of AGG: administration of AGG concentrates, fresh frozen plasma, concentrates containing factor IX; desmopressin (increasing the level of factor VIII);

2) transfusions, venipuncture against the background of the administration of epsilon-aminocaproic acid;

3) prevention of injuries and use of drugs containing aspirin.

Symptoms of diseases of the blood system are quite diverse and most of them are not specific (that is, they can also be observed in diseases of other organs and systems). It is precisely because of the nonspecificity of the symptoms that many patients do not seek medical help in the first stages of the disease, but come only when there is little chance of recovery. However, patients should be more attentive to themselves and if they have doubts about their own health, it is better not to “delay” and wait until it “goes away on its own,” but to immediately consult a doctor.

So, let's look at the clinical manifestations of the main diseases of the blood system.

Anemia

Anemias are a group of syndromes common feature which is a decrease in the level of hemoglobin in the blood. Sometimes anemia is an independent disease (hypo- or aplastic anemia, etc.), but more often it occurs as a syndrome in other diseases of the blood system or other body systems.

There are several types of anemia, common clinical sign which is an anemic syndrome associated with oxygen starvation of tissues: hypoxia.

The main manifestations of anemic syndrome are as follows:

• pallor of the skin and visible mucous membranes ( oral cavity), nail bed;
• increased fatigue, a feeling of general weakness and weakness;
• dizziness, flashing spots before the eyes, headaches, tinnitus;
• sleep disturbances, deterioration or complete absence of appetite, sexual desire;
• increased breathing, feeling of lack of air: shortness of breath;
• palpitations, increased heart rate: tachycardia.

Manifestations iron deficiency anemia are caused not only by hypoxia of organs and tissues, but also by iron deficiency in the body, the symptoms of which are called sideropenic syndrome:

• dry skin;
• cracks, ulcerations in the corners of the mouth - angular stomatitis;
• layering, brittleness, cross-striations of nails; they are flat, sometimes even concave;
• burning sensation of the tongue;
• perversion of taste, desire to eat toothpaste, chalk, ash;
• addiction to some atypical odors: gasoline, acetone and others;
• difficulty swallowing solid and dry foods;
• in females - urinary incontinence when laughing, coughing; in children - ;
• muscle weakness;
• in severe cases - a feeling of heaviness, pain in the stomach.

B12 and folate deficiency anemia characterized by the following manifestations:

• hypoxic or anemic syndrome (signs described above);
• signs of damage to the gastrointestinal tract (disgust for meat food, loss of appetite, pain and tingling in the tip of the tongue, taste disturbance, "varnished" tongue, nausea, vomiting, heartburn, belching, stool disorders - diarrhea);
• signs of damage to the spinal cord, or funicular myelosis (headache, numbness in the limbs, tingling and crawling, unsteady gait);
• psycho-neurological disorders (irritability, inability to perform simple mathematical functions).

Hypo- and aplastic anemia usually begin gradually, but sometimes debut acutely and progress rapidly. The manifestations of these diseases can be grouped into three syndromes:

• anemic (discussed above);
• hemorrhagic (of various sizes - dotted or in the form of spots - hemorrhages on the skin, gastrointestinal bleeding);
• immunodeficiency, or infectious-toxic (persistent fever, infectious diseases of any organs - otitis media, and so on).

Hemolytic anemia outwardly manifests itself as signs of hemolysis (destruction of red blood cells):

• yellow coloration of the skin and sclera;
• an increase in the size of the spleen (the patient notices a formation in the left side);
• increased body temperature;
• red, black, or brown urine;
• anemic syndrome;
• sideropenic syndrome.

Leukemia

This group malignant tumors, developing from hematopoietic cells. The altered cells multiply in the bone marrow and lymphoid tissue, oppressing and replacing healthy cells, and then enter the bloodstream and spread through the bloodstream throughout the body. Despite the fact that the classification of leukemia includes about 30 diseases, their clinical manifestations can be grouped into 3 leading clinical and laboratory syndromes:

• tumor growth syndrome;
• tumor intoxication syndrome;
• hematopoietic suppression syndrome.

Tumor growth syndrome occurs due to the spread of malignant cells to other organs and systems of the body and the growth of tumors in them. Its manifestations are as follows:

• swollen lymph nodes;
• enlarged liver and spleen;
• pain in bones and joints;
• neurological symptoms (persistent severe headache, nausea, unrelieved vomiting, fainting, convulsions, strabismus, unsteadiness of gait, paresis, paralysis, and so on);
• changes in the skin - the formation of leukemides (white tubercles consisting of tumor cells);
• inflammation of the gums

Tumor intoxication syndrome is associated with the release of biologically active substances that are toxic to the body from malignant cells, the circulation of cell decay products throughout the body, and changes in metabolism. Its signs are as follows:

• malaise, general weakness, fatigue, irritability;
• decreased appetite, poor sleep;
• sweating;
• increased body temperature;
• itching of the skin;
• weight loss;
• joint pain;
• renal edema.

Hematopoietic suppression syndrome occurs due to a lack of red blood cells in the bloodstream (anemic syndrome), platelets (hemorrhagic syndrome) or leukocytes (immunodeficiency syndrome).

Lymphomas

Malignant is a group of tumors of the lymphatic system that arise as a result of the formation of pathologically altered lymphoid cells capable of uncontrolled proliferation (reproduction). Lymphomas are usually divided into 2 large groups:

• Hodgkin's (Hodgkin's disease, or lymphogranulomatosis);
• non-Hodgkin's lymphomas.

Lymphogranulomatosis– a tumor of the lymphatic system with primary damage to lymphoid tissue; accounts for about 1% of all adult cancers; Persons aged 20 to 30 and over 50 years of age are more often affected.

Clinical manifestations of Hodgkin's disease are:

• asymmetrical enlargement of the cervical, supraclavicular or axillary lymph nodes (the first manifestation of the disease in 65% of cases); the nodes are painless, not fused to each other or to surrounding tissues, movable; as the disease progresses, the lymph nodes form conglomerates;
• in every 5th patient, lymphogranulomatosis debuts with enlarged mediastinal lymph nodes, which is initially asymptomatic, then cough and chest pain, shortness of breath appear);
• several months after the onset of the disease, symptoms of intoxication appear and steadily progress (fatigue, weakness, sweating, loss of appetite and sleep, weight loss, itchy skin, increased body temperature);
• tendency to infections of viral and fungal etiology;
• all organs containing lymphoid tissue are gradually affected - pain occurs in the sternum and other bones, the liver and spleen increase in size;
• in the later stages of the disease, signs of anemic, hemorrhagic syndromes and a syndrome of infectious complications appear.

Non-Hodgkin's lymphomas is a group of lymphoproliferative diseases with primary localization mainly in the lymph nodes.

Clinical manifestations:

• usually the first manifestation is an enlargement of one or more lymph nodes; when palpated, these lymph nodes are not fused together and are painless;
• sometimes, in parallel with the enlargement of the lymph nodes, symptoms of general intoxication of the body appear (weight loss, weakness, itching of the skin, increased body temperature);
• a third of patients have lesions outside the lymph nodes: in the skin, oropharynx (tonsils, salivary glands), bones, gastrointestinal tract, lungs;
• if the lymphoma is localized in the gastrointestinal tract, the patient is worried about nausea, vomiting, heartburn, belching, pain in the abdomen, constipation, diarrhea, intestinal bleeding;
• sometimes lymphoma affects the central system, which is manifested by severe headaches, repeated vomiting that does not bring relief, convulsions, paresis and paralysis.

Myeloma

Myeloma, or multiple myeloma, or plasmacytoma is separate species tumors of the blood system; comes from precursors of B-lymphocytes that retain a certain ability to differentiate.

Main syndromes and clinical manifestations:

• pain syndrome (pain in the bones (ossalgia), radicular pain between the ribs and in the lower back (neuralgia), pain in the peripheral nerves (neuropathy));
• bone destruction syndrome (bone pain associated with osteoporosis, compression bone fractures);
• hypercalcemia syndrome (increased calcium levels in the blood - manifested by nausea and thirst);
• hyperviscose, hypercoagulability syndrome(due to violation biochemical composition blood - headaches, bleeding, thrombosis, Raynaud's syndrome);
• recurrent infections (due to immunodeficiency - recurring sore throats, otitis, pneumonia, pyelonephritis, and so on);
• renal failure syndrome (swelling that first appears on the face and gradually spreads to the trunk and limbs, increased blood pressure, which cannot be corrected by conventional antihypertensive drugs, cloudiness of urine associated with the appearance of protein in it);
• in the later stages of the disease - anemic and hemorrhagic syndromes.

Hemorrhagic diathesis

Hemorrhagic diathesis is a group of diseases whose common feature is increased bleeding. These diseases may be associated with disorders in the blood coagulation system, a decrease in the number and/or function of platelets, pathology of the vascular wall, and combined disorders.

Thrombocytopenia– decrease in platelet content in peripheral blood less than 140*10 9 /l. The main symptom of this disease is hemorrhagic syndrome varying degrees severity, directly dependent on platelet levels. Usually the disease is chronic nature, but can also be acute. The patient pays attention to pinpoint rashes and subcutaneous hemorrhages on the skin that appear spontaneously or after injuries. Blood leaks through wounds, injection sites, and surgical sutures. Less common are nosebleeds, bleeding from the digestive tract, hemoptysis, hematuria (blood in the urine), and in women, heavy and prolonged menstruation. Sometimes the spleen becomes enlarged.

Hemophilia is a hereditary disease characterized by a blood clotting disorder due to a lack of one or another internal clotting factor. Clinically