Anemia in children Pathology of blood

◼ Anemia ◼ Hemoblastoses - malignant tumors arising from hematopoietic cells) ◼ Hemorrhagic diseases - hemophilia, thrombocytopenic purpura, hemorrhagic vasculitis Pathology of blood

◼ Blood diseases in children 16.6 ‰ of first detected diseases 43.9 ‰ of all diseases ◼ About 1 billion people have deficiency ◼ Hemoblastoses account for 30% of childhood tumors, 80% for acute lymphoblastic leukemia. The composition of the blood ◼ Blood cells 40-45% of blood volume: Plasma ~ 99.9% - erythrocytes ~ 0,1% - leukocytes and platelets Leukocytes ◼ Plasma - 55-60% - Water (92%) - Proteins (7%) - Other substances (1%) Erythrocytes Bone marrow of a child and adult

• in a child, a red (active) bone Active bone marrow is located in all bones marrow of the skeleton, and from 3 to 4 years old, its replacement with fat begins. • in an adult, the red bone marrow is located in the spongy bones of the skeleton and in the epiphyses of the tubular bones. • The mass of red bone marrow is 1400 - 1,500 g Photo of a scanning electron microscope

Erythrocyte Trombocyte Leukocyte Erythrocytes

◼ 99.9% of uniforms ◼ Contains red pigment , which binds and transports O2 and CO2 ◼ It has a disk shape ◼ Diameter → 7.2-7.5 μm ◼ Thickness → 2.5 μm Hemoglobin

◼ The protein consists of 4 polypeptides: 2 α-chains and 2 β- chains ◼ Each chain contains one heme molecule, ◼ The heme iron ion is able to re-bond the oxygen molecule. Anemia

◼ literal translation from Greek - bloodless (anaemia; "an" - without, "haema" - blood). ◼ Anemia is a pathological condition characterized by a decrease in hemoglobin per volume unit of blood ◼ less than 110 g / l in children under 6 years ◼ less than 120 g / l in children older than 6 years Classification of anemia

I. Anemia caused by lack of hematopoietic factors ▪ mainly ▪ mainly ▪ mainly protein deficiency 80% of blood diseases are deficiency anemia 80% of deficiencyanemia is iron deficiency Classification of anemia II. Hypoplastic and aplastic anemia A. Hereditary hypoplastic anemia 1. With the general defeat of a hemopoiesis: Fanconi anemia, anemia of Estern - Dameshek 2. With a selective lesion of erythropoiesis: Blackfen-Daymond anemia B. Acquired hypo- and aplastic anemia Classification of anemia

III. Hemolytic anemia A. Hereditary hemolytic anemia 1. erythrocyte membrane disturbance: hereditary microspherocytosis 2. Impairment of the activity of enzymes RBCs: deficiency of glucose-6-phosphate dehydrogenase activity 3. violation of the structure or synthesis of hemoglobin: thalassemia B. Acquired hemolytic anemia Classification of anemia

IV. Anemia caused by blood loss (post- hemorrhagic): ◼ Acute ◼ Chronic (pathogenesis - iron deficiency).

Mixed Genesis Anemia: A. In acute , sepsis. B. In burns. B. In tumors and leukemia. D. In endocrinopathies. Classification of anemia

By severity: ◼ light (Hb 110-91 g / l); ◼ moderate (Hb 90-71 g / l); ◼ severe (Hb 70 - 51 g / l); ◼ extremely severe (Hb less than 50 g / l). By the degree of erythrocyte saturation with hemoglobin

◼ Hypochromic <0.85 ◼ Normochrome 0.9 - 1.1 ◼ Hyperchromic> 1.1 By the type of regeneration

The number of reticulocytes in ‰ ◼ Regenerative = 4 - 49 ◼ Hyperregenerative> 50 ◼ Hyporegenerative 1 - 3 ◼ Aregenerative = 0 Morphological classification of anemias ◼ I. Macrocytic anemia (MCV> 100 μm (fl); erythrocyte diameter> 8 μm) (vitamin B12 and folic acid deficiency, liver disease, preleukemia).

◼ II. Microcytic anemia (MCV <80 μm (fl), erythrocyte diameter <6.5 μm) (deficiency of iron, impaired synthesis of globin, impaired synthesis of porphyrin and heme).

◼ III. Normocytic anemia (MCV 81-99 μm (fl), erythrocyte diameter 7.2-7.5 μm) (recent blood loss, erythrocyte hemolysis, hypo- and aplastic anemia, myelofibrosis).

All erythrocytes are distinguished by the size: normocytes (d = 7,2-7,5 μm) microcytes (d = 5,5-6,5 μm) macrocytes (d = 8,5-9,0 μm). Normal and abnormal red blood cells

a - normal red blood cells b - megalocytes с- microsephrocytes d - poikilocytes, anisocytes, macrocytes, a c microcytes

b d Iron deficiency anemia (IDA)

◼ First described by Lange in 1554. ◼ WHO defines iron deficiency anemia in 20% of the world's population ◼ In Ukraine, the incidence of IDA varies between 24 and 41.5%. ◼ Iron deficiency is determined in 50-70% of young children and in 20 - 25% of older children. The epidemiology of IDA

◼ WHO: Among the most common diseases, IDA ranks 1st, about 24.8%. ◼ The greatest risk of developing IDA is in children in the first 2 years of life (50- 70%), ◼ in teenagers (25-30%) Functions of iron in the body

◼ Depot and transportation of oxygen ◼ Metabolism: iron is in the composition of more than 70 enzymes that control: cholesterol metabolism, destruction of toxins by the liver; the process of hematopoiesis; DNA synthesis; assimilation of group ◼ Antioxidant protection ◼ Response of the immune system to bacterial or viral ; activation of phagocytosis ◼ Redox reactions; ◼ Cell Energy: Iron is a component of cytochromes ◼ Hormones: iron is required for the production of thyroid hormones, glucocorticoids, androgens ◼ Growth regulation Iron in the body

◼ In the body of an adult, the amount of iron ranges from 3.5 to 5 g, in newborns - 300-400 mg ◼ we lose 1-2 mg every day (women in menstruation period 15-30 mg). With food, with a balanced diet, we receive an average of 10-15 mg of iron every day, and we are only able to absorb 10-15% of this amount. Norms of daily requirement of the person in iron

◼ Children under 6 months - 6 mg; ◼ Children 6 months - 10 years - 10 mg; ◼ Over 10 years - 12-15 mg; ◼ Pregnant women - 19 mg (sometimes up to 50 mg); ◼ Lactating women - 16 mg (sometimes up to 25 mg). The distribution of iron in the body

◼ RBC hemoglobin (60-65%) ◼ Myoglobin (10-14%) ◼ (8-10%) ◼ Hemosiderin (8-10%) ◼ Cytochromes, catalase (5-7%) ◼ Transport iron (0.1-0.2%) Exchange of iron Food iron Fe +++ Stomach hydrochloric acid, ascorbic acid, fructose, cysteine

Small intestine Transferrin of mucous membrane cells

Blood blood plasma transferrin

Ferritin of liver Hemosiderin of liver bone marrow and bone and bone marrow erythroblasts marrow Iron absorption

◼ About 10% of dietary iron is absorbed in the duodenum and in the initial small intestine ◼ With IDA, the suction zone expands distally ◼ Heme iron is absorbed by 20%, non-heme iron by 5-8% ◼ Foods are mainly Fe 3+ ◼ Fe 3+ under the action of HCl is reduced to Fe 2+ ◼ Up to 50% of iron is absorbed from the breast milk, up to 10% from cow's milk Iron deficiency condidtion Consequences of iron deficiency (when reducing iron in brain tissue) 80% of the iron found in the adult brain is deposited in the first 10 years of life ◼ impairment of neuro-psychiatric functions in children

◼ decrease in intellectual development (IQ)

◼ slowing down of logical thinking, speech, impaired learning, deviation in the psyche. Consequences of iron deficiency

◼ reduction of glucocorticoid and androgenic function of the adrenal glands, ◼ degenerative-dystrophic changes of skin epithelium, gastrointestinal mucosa, respiratory tract, ◼ decreased activity of gastric and pancreatic enzymes, impaired absorption of amino acids, vitamins, trace elements, ◼ development of enteropathy and malabsorption syndrome. Reasons for IDC

◼ Insufficient supply of iron with food; ◼ Violation of iron absorption in the intestine; ◼ Increased iron loss (chronic bleeding); ◼ Increased need of iron during intensive growth and development of the baby; ◼ Vitamin and micronutrient deficiency Reasons for IDC

◼ Insufficient iron intake with food (early artificial feeding, especially non-adaptated formula, vegetarian diet, etc.); ◼ Increased need of iron in children (preterm; those born with a large body weight; children of the 2nd half and 2 years of age, prepubertal and pubertal age); Reasons for IDC

◼ Increased loss of iron in bleeding, impaired intestinal absorption (malabsorption syndrome, chronic bowel disease); ◼ Disorders of iron metabolism in the body due to hormonal changes, impaired iron transport with insufficient activity or decrease in the transferrin content in the body. Stages of IDC

◼ Prelatent iron deficiency (depot depletion) ◼ Latent iron deficiency (reducing the content of both deposited iron and transport pool without reducing hemoglobin and the development of anemia) ◼ Iron deficiency anemia (IDA) Iron deficiency condition

Norma Reduced Iron- Iron- iron deficiency deficiency reserves erythropoesis anemia iron depot

transport iron

erythron iron

bone marrow depot iron binding ability of transferrin, µmol / l serum ferritin, µkg/l , µkg/l transferrin saturation,% Protoporphyrin of erythron RBC Normal Normal Normal Hypochromic, mycrocytes, Iron deficiency anemia Clinically manifested IDC 1). depleted iron stores in the body; 2). hemoglobin concentration decreases and anemic hypoxia develops; 3).the activity of tissue respiration enzymes is inhibited, dystrophic processes in the tissues develop. IDA Clinic

◼ hemic hypoxia (common anemic syndrome) ◼ sideropenic syndrome ◼ metabolic intoxication Clinical signs of anemic syndrome The severity of the clinic does not depend on the severity of the anemia, but on the duration of the disease, adaptation to hypoxia ◼ Astheno-neurotic syndrome (fainting, adynamia, decreased intelligence and emotionality, retardation in psychomotor development). ◼ Hypoxic syndrome - general malaise, dizziness, headaches, muscle aches. ◼ Cardiovascular syndrome - ↑ heart rate, attenuation of heart tones, functional systolic murmur. ◼ Respiratory system changes - tachypnea. ◼ Prolonged subfebrile temperature ◼ Reduced appetite or lack of appetite. ◼ Increase in lymph nodes (29%) ◼ ↓ local immunity Signs of sideropenic syndrome

◼ Epithelial changes (dystrophic changes of skin and its appendages). Skin dryness, dullness, hair loss, brittleness, nail layering. There may be angular stomatitis ◼ Change in taste and smell. Children start eating chalk, clay, sand; eat raw foods that require culinary processing. Characteristic passion for unusual odors: kerosene, gasoline, acetone. ◼ Astheno-vegetative reactions ◼ Dyspepsia ◼ Intestinal absorption disorders ◼ Muscle weakness → urinary incontinence during coughing, enuresis IDA clinic in early age children ◼ the striking pallor of the skin at still normal levels of erythrocytes and hemoglobin; ◼ The “unreasonable” stopping of weight gain; ◼ "Unreasonable" loss of appetite; ◼ frequent ARVI ◼ 10% of children have hepatopathic palpation or splenomegaly. IDA clinic in older children

◼ dry skin; flaking of skin on elbows and knees ◼ hair dullness and brittleness; ◼ atrophy of the papillae of the tongue ("varnished" tongue), cracks of the tongue, painful cracks in the corners of the mouth (angular stomatitis) ◼ severe asthenic syndrome Epithelial changes Metabolic intoxication

Generalized tissue hypoxia results in ejection of biologically active substances: histamine, serotonin, heparin clinically: ◼ rapid fatigue, ◼ reduced memory, ◼ headache (in the evening), ◼ hypotension, ◼ subfebrile temperature, ◼ astheno-vegetative syndrome. Laboratory criteria of IDA

◼ microcytosis; ◼ anisocytosis and poikilocytosis; ◼ Hypochromia of RBC; ◼ basophilic granularity of erythrocytes; ◼ normoblastosis; ◼ reticulocytes are normal or enlarged Laboratory indicators that characterize the state of iron metabolism

◼ Serum iron (SI) - in newborns 5.0-19.3 µmol / l; in children older than 1 month. - 10.6-33.6 μmol / l. ◼ Total iron binding ability of serum (TIBAS) - characterizes the total amount of iron that can be contacted with plasma transferrin. ◼ The norm of the TIBAS is 40.6-62.5 μmol / l ◼ Latent iron binding ability of serum (LIBAS) is an indicator that reflects the mathematical difference between the values of TIBAS and SI: LIBAS = TIBAS - SI ◼ Normally> 47 µmol / L. Treatment of anemia

◼ Diet therapy ◼ Ferrotherapy (at Hb <100 g / l) ◼ Enzymatic therapy ◼ Vitamin therapy Iron preparations

◼ preparations of bivalent iron - iron sulfate (12-16%), gluconate (20-22%), chloride (5-6%), fumarate (14-16%), lactate (7-9%) succinate, etc .; ◼ preparations of trivalent iron - iron hydroxide in the form of polymaltose or sucrose complex. It is advisable to prescribe divalent iron preparations due to its optimum absorption Classification of iron preparations I. Monocomponents: Iron sulphate: Hemofer prolongatum, Ferrogradumed, Conferon. Fumarate iron: Heferol. Iron Chloride: Hemophore. II. Combined: With folic acid: Ferromed, Fefol. With serine: Actiferin. With ascorbic acid: Sorbifer durulex, Ferroplex. With ascorbic acid and mucoprotease: Tardiferone, Hypotardiferon (with folic acid). With folic acid, calcium, vit. C and group B: Vifer, Natabsk. With folic acid and amino acids: Irravite, Irradian. With Vit. group B and nicotinic acid: Fesovit .. Calculation of daily dose of iron preparations

◼ for children under 3 years - 3-5 mg/kg/day of elemental iron (up to 25 mg / day), ◼ 4-6 years - 50-70 mg/day ◼ 7 years and older - up to 100 mg/day Rules of ferrotherapy

◼ Treatment for IDA should start with a dose of 1/2-1/4 from therapeutic gradually bringing it to full therapeutic (7-14 days). ◼ Take at intervals between meals, preventing the formation of insoluble salts that are unable to absorb the components of the food. ◼ It is advisable to combine with the appointment of ascorbic acid (0.1 g), which increases the absorption of iron. Treatment effectiveness criteria ◼ development of a reticulocytic crisis on day 12-14 of treatment (increase of reticulocytes to 20-100 ‰); ◼ normalization of morphological features of erythrocytes; ◼ daily increase of Нb on 2 g / l and more; ◼ normalization of laboratory signs of iron balance in the body; ◼ improvement or normalization of ECG indicators; ◼ the disappearance or significant attenuation of noises in the heart; ◼ improvements in the clinical picture: muscle weakness, memory improvement, limb paresthesia, etc.. Diet for anemia

NO! Iron content in food

Product name Iron content(mg/100 g) ◼ Beef liver 9,0 ◼ Beef tongue 5,0 ◼ Rabbit meat 4,4 ◼ Beef (young) 4,0 ◼ Turkey meat 4,0 ◼ Chicken meat 3,0 ◼ Sturgeon caviar 3,0 ◼ Beef (old) 2,8 ◼ Eggs 2,7 ◼ Mackerel 2,3 ◼ Carp 2,2 ◼ Lin 1,4 Iron content in food

The name of the plant product Iron content (mg/100 g) ◼ Seaweed 16,0 ◼ Oatmeal 10,7 ◼ Buckwheat 7,8 ◼ Oat flakes 7,8 ◼ Peaches 4,1 ◼ Quince 3,0 ◼ Pears 2,3 ◼ Apples 2,2 ◼ Plums 2,1 ◼ Apricot 2,1 ◼ Cherry 1,4 ◼ Cauliflower 1,4 Diet for anemia

◼ Dairy products should be taken in a minimum amount (no more than 400-500 ml / day), since calcium of milk forms soluble iron salts. ◼ It is necessary to limit the flour products, because the phytin they contain complicates the absorption of iron. ◼ Drinking beverages containing tannin (tea, coffee) leads to a clear reduction in the absorption of iron from food. Blood Transfusion

10-15 mg / kg, older children - 150-250 ml ◼ erythrocyte mass or washed erythrocytes ◼ hemoglobin level of 40-70 g / l in combination with signs of disorders of central hemodynamics, hemorrhagic shock, anemic coma, hypoxic syndrome ◼ the effect is short-term Vitamin deficiency anemia

Erythro-, and hranulo- trombotsytopoesis are violated. Occurs when: ◼ celiac disease, ◼ chronic gastrointestinal diseases, ◼ chronic hemolytic process, ◼ when using anticonvulsants, ◼ with nutritional deficiency (goat's milk in a baby's diet), ◼ chronic liver and kidney diseases, ◼ in premature infants. Diagnostic criteria for B12-deficiency anemia

◼ General anemic syndrome ◼ Gastroenterological syndrome (↓ appetite, glossitis, epigastric gravity, achlorhydria, unstable bowel movements) ◼ Neurological syndrome (funicular myelosis): impaired sensitivity of the extremities, changes in gait and coordination of movements, numbness of the lower extremities) ◼ There is no sideropenic syndrome Folic deficiency anemia

◼ Occurs less frequently than B12 deficiency ◼ Causes: prematurity, goat milk feeding, vegetarianism, malabsorption syndrome ◼ The reserve of folic acid in the body for 2-3 months ◼ Contained in all products, destroys when heated ◼ Proteins are not required for folic acid absorption Laboratory indicators of vitamin deficiency anemia

◼ Erythrocyte hyperchromia ◼ Basophilic infiltration of red blood cells, ◼ Macrocytosis ◼ Erythrocytes with Jolly bodies, Kebot rings and nuclear forms of erythroblasts (megalocytes and megaloblasts). Treatment of vitamin deficiency anemia

◼ Vitamin B12 50-100 mcg every other day, 10-15 injections ◼ folic acid 0.25 mg (¼ tab.) - 2 mg (2 tab.) per day Protein deficiency anemia develops as a result of starvation or predominantly carbohydrate feeding of children Protein deficiency anemia is always accompanied by a lack of other hematopoietic factors and is “pandeficiency”. Protein deficiency anemia

Clinic: ◼ expressed general degenerative changes, ◼ signs of polyhypovitaminosis ◼ disorders of pigment metabolism (dyschromia of skin and hair, their fragility is increased), ◼ blepharitis, ◼ pasty tissue. ◼ , vomiting, diarrhea, ◼ enlargement and consolidation of the liver. Protein deficiency anemia

◼ Normally regenerating anemia, mainly normocytic. ◼ Erythrocyte life expectancy is reduced by 2 times (without signs of hemolysis). ◼ Blood biochemistry: hypoproteinemia, hypoalbuminemia, dysproteinemia. Hemolytic anemia

Group of hereditary and acquired diseases, the main feature of which is increased destruction of erythrocytes and their life shortening from 90-120 days to 12-14 days Hemolysis can be: ◼ intracellular (RES cells - spleen) ◼ intravascular Hemolytic anemia

Triad of hemolytic anemia: ◼ Hepatolyenial syndrome ◼ Jaundice (lemon-yellow) hyperbillirubinemia due to indirect fraction ◼ Anemia (normochromic, hyperregenerative) general anemic syndrome In intravascular hemolysis: ◼ Hemoglobinemia ◼ Hemoglobinuria and hemosiderinuria (red or black urine) ◼ Hemosiderosis of internal organs ◼ Tendency to microthrombosis Classification of hemolytic anemias

Hemolytic anemias

Hereditary (congenital) Acquired Hemolytic disease of newborns Erythrocytopathy when transfusing incompatible blood under the action of drugs Fermentopathy (sulfonamides) with viral infections, the effects of physical and chemical substances, hemolytic poisons Hemoglobinopathies under the influence of mechanical factors, physical activity Laboratory indicators of hemolytic anemia

◼ lower number of RBC and hemoglobin, ◼ normal CI (sometimes ↓), ◼ high reticulocytosis (up to 100 ‰), ◼ impaired osmotic resistance of erythrocytes ◼ erythrocytes of a specific form (spherocytes, sickles, etc.). ◼ hyperbilirubinemia due to the indirect fraction Minkowski-Schoffar disease (hereditary microspherocytosis) Hereditary disease, caused by a defect of membrane proteins in erythrocytes which become spherical forms with the next their destruction by macrophages of the spleen Pathogenesis of Minkowski- Schoffar disease

◼ a defect in the proteins of the erythrocyte membrane, which is accompanied by increased permeability and the entry of a significant amount of sodium ions into the cells. ◼ accumulation of water in the cells which causes red blood cells to become spherical.

Minkowski-Schofar Disease Clinic triad of symptoms: jaundice, anemia, splenomegaly ◼ The disease can occur for a long time in a latent form, in which the main symptom of the disease (anemia) is absent. ◼ Moderate anemia (Hb 80 - 100 g / l) is maintained as a constant symptom or becomes particularly pronounced (Hb 58 - 66 g / l) during the period of intensive erythrocyte destruction (hemolytic crises). ◼ A common symptom is jaundice, which is associated with an increase in the content of indirect bilirubin in the blood Laboratory diagnostics of Minkowski-Schoffar disease

◼ microspherocytosis of erythrocytes ◼ reticulocytosis up to 50 ‰ ◼ reduction of osmotic resistance of erythrocytes against hypotonic solutions of sodium chloride ◼ (norm 0.44-0.48% - 0.28-0.32% NaCl). ◼ During the hemolytic crisis, the free fraction of serum bilirubin is significantly increased. Treatment and prognosis

◼ Treatment during hemolytic crisis is aimed at eliminating anemia, hypoxia, hyperbilirubinemia. ◼ The method of choice in the treatment of hereditary spherocytosis is splenectomy, optimal at the age of 4-5 years. Splenectomy provides a practical recovery, despite the persistence of spherocytosis. Fermentopathies

Glucose-6-phosphate dehydrogenase deficiency ◼ ↓ the activity of glycolysis enzymes leads to increased sensitivity of erythrocytes to oxidative stress ◼ Hemolysis is provoked by medicines, viral infections ◼ Clinic depends on the degree of enzyme deficiency (jaundice, darkening of the urine) ◼ Diagnosis: genetic screening ◼ Treatment: avoid provocative factors, in severe hemolysis - substitution therapy Hemoglobinopathies

Mutations of coding genes amino acid sequences and synthesis of globin chains ◼ Thalassemia (α- and β-thalassemia) ◼ Sickle cell anemia ◼ Hemoglobinopathy C, E. Thalassemia

◼ inherited by autosomal recessive type ◼ ↓ synthesis of globin chains ◼ ineffective erythropoiesis ◼ hypochromic microcytic anemia ◼ excess of α- or β-globin chains → ↓ life expectancy of erythrocytes → hemolysis, cholelithiasis, splenomegaly ◼ body overload with iron α-thalassemia Depending on the number of mutant genes can be: ◼ Asymptomatic carrier: in the defeat of 1 of 4 genes responsible for the formation of α-chains of globin. the formation of α-chains is sufficient for the synthesis of normal Hb. There are no clinical manifestations ◼ Small α-thalassemia: defect of 2 genes encoding the formation of α- chains of globin. In this case, α-chains are not formed enough → the formation of normal Hb is limited. Clinical manifestations are weak. ◼ Hemoglobinopathy H. A severe form of the disease, the defeat of 3 genes out of 4. Α-globin chains are formed in small quantities → almost all Hb of red blood cells are composed of four b-chains. The disease is characterized by a long and difficult course. ◼ Intrauterine dropsy: damage to all 4 genes responsible for the formation of α-chains of globin. In this form of the disease, the death of the fetus occurs in the pre-natal period or immediately after birth. β-thalassemia

Depending on the number of mutant genes distinguish: ◼ Great Thalassemia (Cooley anemia). Severe form of the disease, characterized by damage to both genes that encode the formation of b-chains of globin. ◼ Intermediate thalassemia. It is characterized by mutation of one or both genes, but b-globin chains that are formed enough to synthesize a certain amount of normal Hb. Clinically less pronounced. ◼ Small b-thalassemia. It is characterized by mutation of one gene, which leads to a slight decrease in the number of b-chains of globin. The course of the disease without clinical manifestations. Epidemiology Fermentopathy and hemoglobinopathy are widespread in countries with high rates of malaria (Mediterranean, Southeast Asian countries) Diagnosis of thalassemia

◼ ◼ hyperregenerative anemia ◼ erythrocytes of various shapes in the smear of blood; ◼ normocytes ◼ fetal hemoglobin is increased in red blood cells. ◼ in biochemical analysis of blood hyperbilirubinemia due to free fraction, hypersideremia; Treatment of hemolytic anemias

◼ Detoxification therapy: infusion therapy with solutions of 5-10% glucose with insulin, 0.9% NaCl solution, cocarboxylase, ascorbic acid. If necessary - in conjunction with infusion therapy in some cases use plasmaphoresis. ◼ Antispasmodics and choleretic drugs: no-spa, allohol, holosas, 25% solution of magnesium sulfate. ◼ Phenobarbital has a bilirubin-conjugating effect, inducing liver glucuronyltransferase activity of 10-15 mg / kg for 7- 10 days. ◼ Antioxidants: Vitamin E. Thalassemia treatment

1. Red blood cell transfusion so that Hb content is not fell to low numbers, to repeat transfusion at its levels - 95-100 g / l. 2. Removal of excess iron from the body: desferal, ejidzad, vit. C 3. Reduction of urate diathesis: allopurinol 4. Splenectomy in severe forms 5. Bone marrow transplantation is the only method radical treatment Sickle cell anemia ◼ hereditary hemoglobinopathy associated with impaired structure of the protein Hb, in which it acquires a special crystalline structure (Hb S). ◼ The clinic manifests itself in a homozygous state: - hemolytic anemia - vaso-occlusive crises (provoking infections, physical or emotional exertion) are manifested by painful attacks, heart attacks - increased susceptibility to infections ◼ Diagnosis: sickle test, ◼ genetic analysis Aplastic anemia

A group of diseases caused by a defect in the stem cell or its microenvironment, which leads to a decrease or lack of production of hematopoietic cells Clinically: ◼ sharply expressed hemorrhagic syndrome ◼ septic-necrotic processes

Aplastic anemia

◼ Pancytopenia ◼ Reticulocytes are undetectable or decreased Aplastic anemia Plt

RBC

Bone marrow WBC Causes of aplastic anemia

◼ Contact with toxic substances ◼ Irradiation ◼ Infections ◼ Genetic predisposition ◼ Use of some medicines ◼ If it is not possible to identify the causes of the disease, talk about idiopathic aplastic anemia Fanconi anemia

hypoplastic anemia with general lesion of hematopoiesis and hereditary developmental abnormalities ◼ inheritance is autosomal recessive ◼ pathogenesis - impaired stem cell DNA repair processes ◼ anemia appears clinically after 4-10 years ◼ in the bone marrow a significant decrease in the number of nuclear cells of the myeloid row. ◼ in peripheral blood pancytopenia (except lymphocytes) Fanconi Anemia (Clinic) skin pigmentation, deformity of the skeleton (absence or shortening of the thumb, underdevelopment of the radial bone, congenital dislocation of the thigh, cervical rib, claw-foot). neurological disorders (strabismus, underdevelopment of one or both eyes, lowering of the eyelid, eye tremor, deafness, mental retardation), lesions of the genitals (underdevelopment of the genitals, absence of one or both testicles, hypospadias), kidney defects (underdevelopment of kidneys, horseshoe kidney, multiple cysts in kidney tissues), congenital heart defects with the development of anemia - signs of hypoxia, thrombocytopenia (bleeding), leukopenia (infection). Estren's – Dameshek anemia

◼ Hereditary hypoplastic anemia with a general lesion of hematopoiesis without hereditary anomalies of development ◼ It is characterized by progressive bone marrow hypoplasia and pancytopenia in the peripheral blood. Blackfan-Diamond anemia

characterized by a selective lesion of the erythroid row ◼ authentically unexplored type of inheritance (autosomal dominant type of inheritance is assumed, occurs in 25% of patients) ◼ clinic of severe anemia from the first year of life ◼ treatment: hemotransfusion, glucocorticoids Treatment of aplastic anemia

◼ Termination of contact with the provoking factor in the case of acquired aplastic anemia ◼ Substitution therapy (erythromass, thromboconcentrate) ◼ GM-SCF, G-SCF growth factors ◼ Antilymphocytic immunoglobulin ◼ Immunosuppressive therapy - steroids, cyclosporine ◼ Symptomatic therapy (hemostatic, antibacterial therapy) ◼ Bone marrow transplantation June 14 marks World Donor Day, dedicated to the birth of Karl Landsteiner, an Austrian scientist, who first divided the blood into groups.

The word "donor" comes from lat. donare - «donate» Every donor is a hero! Every third inhabitant of the Earth will have to do blood transfusions at least once in his life Active donors are less likely to suffer from cardiovascular disease and can more easily tolerate blood loss in road accidents and other accidents. According to WHO, people who donate blood regularly live on average 5 years longer, because they have increased blood production and regular stimulation of immunity. The world's most famous blood donor, during his lifetime, has donated about 500 liters of blood 624 times. In order to provide sufficient blood for medical purposes, there should be at least 40 donors per 1,000 people in the country. 10- 15% of the population can be donors, but in reality, people who donate blood are ten times less.

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