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IRON-DEFICIENCY ANEMIA
IDA is a disorder in which the iron content in the blood serum, bone marrow and depot decreases, which leads to disruption of the formation of HB and red blood cells, the occurrence of anemia and trophic disorders in tissues.
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REASONS FOR WAITING.
1. Chronic blood loss 2. Increased iron consumption 3. Nutritional iron deficiency 4. Impaired absorption of iron 5. Redistribution of iron deficiency 6. Impaired iron transport with hypo-, atransferrinemia
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DIAGNOSTICS
UAC: Hemoglobin, color index, red blood cells decrease (to a lesser extent). The shape and size of red blood cells change: poikilocytosis ( different shape erythrocytes), microcytosis, anisocytosis (of unequal magnitude). Bone marrow: generally normal; moderate hyperplasia of the red sprout. Special staining reveals a decrease in sideroblasts (erythrokaryocytes containing iron). Biochemistry. Definition serum iron(reduced). Normally 11.5-30.4 µmol/l in women and 13.0-31.4 in men. This analysis is very important, but errors in the determination are possible (not clean test tubes), so normal level syv. iron does not yet exclude IDA. Total iron binding capacity of serum (TIBC) – i.e. the amount of iron that can be bound by transferrin. The norm is 44.8-70 µmol/l. With IDA, this figure increases.
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TREATMENT
Rational treatment of IDA involves a number of principles: 1. IDA cannot be treated with diet alone 2. Compliance with the stages and duration of treatment - relief of anemia - restoration of iron depots in the body The first stage lasts from the start of therapy until the normalization of hemoglobin (4-6 weeks), the second stage is therapy “saturation” – 2-3 months. 3.Correct calculation of the therapeutic dose of iron
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VITAMIN B12 DEFICIENCY ANEMIA
This anemia was first described by Addison and subsequently by Birmer more than 150 years ago (1849), and is accordingly known under the name of these two researchers. At the beginning of the 20th century, this anemia was one of the most frequent illnesses blood that is not amenable to any therapy - hence another name - pernicious or pernicious anemia.
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CAUSES OF VITAMIN B12 DEFICIENCY IN THE BODY
1. Malabsorption 2. Competitive consumption of B12 3. Reduced reserves of vitamin B12 4. Lack of food 5. Lack of transcobalamin-2 or the development of antibodies to it (rarely).
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Damage to the gastrointestinal tract.
First of all, glossitis is typical, according to the author’s description – Günther’s: red lacquered, crimson tongue. It is not detected in everyone - in the presence of a significant and long-term deficiency of vitamin B12 (10-25%). Some patients may have less pronounced manifestations of glossitis - pain in the tongue, burning, tingling, in certain cases inflammation, formation of erosions. Objectively, the tongue has a crimson color, the papillae are smoothed, and there are areas of inflammation at the tip and edges. Other gastrointestinal lesions include atrophic gastritis, which may also be a consequence of vitamin B12 deficiency.
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Damage to the nervous system
Most often affected peripheral nerves, then the rear and side pillars spinal cord. Symptoms appear gradually, starting with peripheral paresthesia - tingling, numbness of the legs, a crawling sensation in the lower limbs; then stiffness of the legs and unsteadiness of gait appear. In rare cases, they are involved upper limbs, the sense of smell and hearing are impaired, and mental disorders, delirium, hallucinations. Objectively, loss of proprioceptive and vibration sensitivity and loss of reflexes are detected. Later, these disturbances increase, the Babinski reflex appears, and ataxia occurs.
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DIAGNOSTICS
UAC. Increase color index(more than 1.1) and MCV. The size of red blood cells is increased, there may be megaloblasts, i.e. hyperchromic and macrocytic anemia. Anisocytosis and poikilocytosis are characteristic. In erythrocytes, basophilic puncture is detected, the presence of remnants of nuclei in the form of Joly bodies and Cabot rings. Leukocytes, platelets and reticulocytes change. Leukocytes - the number decreases (usually 1.5-3.0 10), the segmentation of neutrophils increases (up to 5-6 or more). Platelets – moderate thrombocytopenia; As a rule, hemorrhagic syndrome does not occur. Reticulocytes – the level is sharply reduced (from 0.5% to 0).
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Sternal puncture– is crucial in diagnosis. It must be carried out before starting the administration of vitamin B12, because normalization of bone marrow hematopoiesis occurs within 48-72 hours after the administration of adequate doses of vitamin B12. In the cytogram bone marrow megaloblasts are detected (large atypical cells with a peculiar morphology of the nucleus and cytoplasm) varying degrees maturity, which allows morphological confirmation of the diagnosis. The ratio L:Er = 1:2, 1:3 (No. = 3:1, 4:1) due to severe pathological hyperplasia of the red sprout. There is a pronounced disruption of the maturation and death of megaloblasts in the bone marrow, there are no oxyphilic forms, so the bone marrow looks basophilic - “blue bone marrow”.
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TREATMENT OF B12-DEFICIENCY ANEMIA
The course of treatment consists of daily intramuscular injections of vitamin B12 of 500 mcg, 30-40 injections per course. Subsequently, maintenance therapy of 500 mcg is recommended once a week for 2-3 months, then 2 times a month for the same period. According to the recommendations of American hematologists, maintenance therapy should be carried out for life – 250 mcg once a month (or course treatment 1-2 times a year, 400 mcg/day for 10-15 days).
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Hemolytic anemia
a group of diseases in which there is a shortening of the lifespan of red blood cells, i.e. hemorrhage prevails over blood formation.
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ACQUIRED HEMOLYTIC ANEMIA
Most often worn immune mechanism: Most a common option are autoimmune hemolytic anemia. In this case, antibodies are produced to their own unchanged antigen of red blood cells. The reason is a disruption of natural immunological tolerance, due to which one’s own antigen is perceived as foreign. Autoimmune G.A. may be symptomatic or idiopathic.
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Laboratory characteristics. UAC: anemia in most cases is not severe (Nb decreases to 60-70 g/l), but with acute crises there may be lower numbers. Anemia is often normochromic (or moderately hyperchromic). Reticulocytosis is noted - initially slight (3-4%), upon recovery from the hemolytic crisis - up to 20-30% or more. Changes in the size of red blood cells are observed: macrocytosis, microcytosis, the latter being more characteristic. The number of leukocytes is moderately increased (up to 20+10 9/l), with a shift to the left (leukemoid reaction to hemolysis). Biochemistry of blood. Slight hyperbilirubinemia (25-50 µmol/l). The proteinogram may show an increase in globulins.
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Treatment. The main drug is prednisolone. Prescribed 1 mg/kg per day. If after 3 days there is no effect, the dose is doubled. If administered intramuscularly, the dose is also doubled, intravenously – 4 times more. The positive effect is usually in 90% of cases and above. After stopping hemolysis, the dose is gradually reduced. However, when the dose of prednisolone is reduced, relapses are often observed. If anemia cannot be controlled within 6 months, splenectomy is indicated. The measure is effective - cure in 70-80% of cases. If the result is negative, cytostatics are used (azathioprine, cyclophosphamide).
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APLASTIC ANEMIA
The term "hypoplastic or aplastic anemia" means panhypoplasia of the bone marrow, accompanied by leukocytopenia and thrombocytopenia.
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Bone marrow aplasia can be caused by various factors
Physical (ionizing radiation, high frequency currents, vibration -5%) Chemical (benzene, mercury, pesticides, paints - 60%) Medications (chloramphenicol, macrolides, sulfonamides, analgin, etc. - 32%) Infectious ( viral hepatitis, flu, sore throat... 28%) Other (8%).
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Laboratory data
Anemia is usually normochromic-normocytic. The leukocyte count is usually less than 1.5*109/l (granulocytopenia). Platelets are also reduced. The content of reticulocytes is also reduced. Serum iron content is increased. Bone marrow. Picture of hypo- and aplasia of the bone marrow: decrease in the erythroid (megakaryocytes) and granulocyte series (myelokaryocytes). It is necessary to perform a trepanobiopsy.
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ANEMIA ASSOCIATED WITH CHRONIC DISEASES OF INTERNAL ORGANS (Symptomatic).
HYPOPROLIFERATIVE anemia – anemia characterized by the inability of the red hematopoietic germ to increase the erythroid mass according to the degree of anemia.
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I.A. Novikova
Aplastic anemia
Aplastic anemia (synonymous with malignant aleukia) is a sharp inhibition of all three germs of hematopoiesis in the absence of signs of tumors of hematopoietic tissue.
First described by Ehrlich in 1888 as hemorrhagic diathesis on the background nervous depression hematopoiesis.
A polyetiological disease caused by exogenous and endogenous factors. There are hereditary and acquired ones.
Etiological factors of acquired aplastic anemia:
Chemical factors (benzene, mercury vapor, acids, varnishes, dyes, etc.)
Physical factors (ionizing radiation)
Medicines (cytostatics, antibiotics, anticonvulsants)
Infections, especially viral ones
Endocrine factors, such as hypothyroidism
Benign tumors of the thymus gland
Intensive hematopoiesis (hypoplastic crisis in hemolytic anemia)
Displacement of normal hematopoiesis in leukemia, tumor metastases in the BM
Autoimmune, isoimmune mechanisms
Inhibition of erythropoietin synthesis
Splenogenic pancytopenia
The reasons for the development of about 50% of aplastic anemia are not clear.
states
hematopoiesis
Pathomorphological substrate – general inhibition of hematopoiesis – panmyelopathy. Presumably, under the influence of unfavorable factors (or immune factors?), the ancestral stem cell or its hematopoietic microenvironment (provides regulation of functions and division of the stem cell) → hypoplastic anemia → progression of the process → involvement of all germs - pancytopenia develops.
Sometimes only one germ is involved in the process - partial red cell aplasia - erythroblastophthisis. In this case, anti-erythrocyte antibodies can be detected (usually G, in some A).
hypoplastic
Weakness, pallor, decreased performance, hemorrhagic diathesis of multiple localization, septic complications. On examination: increased heart rate, dilatation of the heart, muffled sounds, systolic murmur mainly at the apex of the heart. Ultrasound already in the first months of the disease shows a slight enlargement of the liver, an increase in the acoustic density of the parenchyma and the heterogeneity of its echostructure. There is no enlarged spleen in AA. If splenomegaly is detected, the diagnosis should be revised.
To confirm the diagnosis, BM examination and trephine biopsy are required.
The prognosis of the disease is worse the older the patient is. The main treatment is CM transplantation.
Laboratory indicators for hypoplastic anemia
Peripheral blood:
Normal-rarely hyperchromic anemia
Aniso- and poikilocytosis is minor
There are no reticulocytes, with an immune nature there is slight reticulocytosis
Leukopenia is persistent with neutropenia (even with the addition of a secondary infection), relative eosinophilia and/or lymphocytosis.
Thrombocytopenia, platelet macrocytosis, thrombocytopathy
ESR (up to 30-50 mm/hour)
Serum iron (N)
aplastic
Myelogram: CM is poor in nucleated elements, delayed maturation of erythro-, leuko- and thrombocytopoiesis cells. ↓↓↓number of megakaryocytes (not detected in severe AA). ↓ neutrophil maturation index (N 0.6-0.8, in patients 0.9 to 6.6). The total content of cellular elements of the neutrophil series decreases, and the percentage of lymphocytes and plasma cells increases. There is a delay in the maturation of erythro- and normoblasts. Sideroblasts even with normal content iron in blood serum.
Trepanobiopathic examination allows us to make a final diagnosis. In TAA, red BM is almost completely replaced by fat.
International criteria for assessing the severity of aplastic anemia
aplastic es-coy anemia
Not severe
Index
peripheral
Hematocrit number< 0,38
Neutrophil cells< 2,5 109/л
Neutrophil cells< 0,5 109/л
Platelets<20 109/л Ретикулоцитов < 1,0%
Index
bone
Varying degrees of decrease in bone marrow cellularity
Significant
decline
cellularity of bone marrow, myeloid cells
Differential
AA diagnosis
Acute leukemia (blasts, splenomegaly, lymphadenopathy)
Agranulocytosis (infection, there is practically no reduction of the red sprout of the bone marrow, the number of megakaryocytes does not decrease).
Subleukemic myelosis (hepato- and splenomegaly, myeloid metaplasia and fibrosis of organ parenchyma, blast elements in the PC and CM)
Myelodysplastic syndrome (dyserythropoiesis, dysgranulo- and dysthrombocytopoiesis)
Paroxysmal nocturnal hemoglobinuria (hemoglobinuria, hemosiderinuria; free bilirubin and Hb in plasma)
Fanconi aplastic anemia
The most common hereditary form of the disease.
General damage to hematopoiesis + developmental anomalies (absence or hypoplasia of the thumb, absence or underdevelopment of the radius, microphthalmos, endocrine disorders, pigmentation of skin areas protected from light - armpits, etc.
Peripheral blood: pancytopenia, normochromic anemia, less often hyperchromic (and then macrocytosis), pronounced mixed poikilocytosis, basophilic granularity, no reticulocytes, leukopenia, thrombocytopenia, hemorrhagic syndrome, ESR - 30-80 mm/hour.
CM point: progressive decrease in cellular elements, the number of plasma cells and basophils may be increased.
In the advanced stage - devastation of the BM (panmyelophthisis).
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Presentation on the topic: ANEMIA
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ANEMIA is a clinical and hematological syndrome characterized by a decrease in the total amount of hemoglobin per unit volume of blood (often with a parallel decrease in the number of red blood cells). ANEMIA is a clinical and hematological syndrome characterized by a decrease in the total amount of hemoglobin per unit volume of blood (often with a parallel decrease in the number of red blood cells). All anemias are considered secondary. Anemic syndrome can be clinically leading or moderately severe. In addition to the circulatory-hypoxic syndrome common to all anemias, each anemia has its own specific symptoms.
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Immune, endocrine and nervous mechanisms are involved in the regulation of erythropoiesis. Immune, endocrine and nervous mechanisms are involved in the regulation of erythropoiesis. Erythropoiesis is influenced by heredity and environmental factors. Normal erythropoiesis is possible if the body has sufficient amounts of amino acids, iron, vitamins B1, B2, B6, B12, C, folic acid, microelements Co, Cu, and other substances. Erythroposis is activated by erythropoietinogen, synthesized in the liver, erythrogenin of the juxtaglomerular apparatus of the kidneys, and the local hormone of erythropoiesis, erythropoietin. Stimulate the production of erythropoietin - ACTH, corticosteroids, growth hormone, androgens, prolactin, vasopressin, thyroxine, insulin. Erythropoiesis is inhibited by estrogens and glucagon.
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Cells of pathological regeneration of erythrocytes, arising from a violation of erythropoiesis. Cells of pathological regeneration of erythrocytes, arising from a violation of erythropoiesis. Megalocyte, megaloblast; red blood cells with Jolly bodies and Cabot rings; erythrocytes with basophilic granularity. Anisocytosis is a pathology of the size of erythrocytes: Normally, the diameter of an erythrocyte is 7.2-7.5 microns; Microcytes - less than 6.7 microns; Macrocytes – more than 7.7 microns; Megalocytes (megaloblasts) – more than 9.5 microns; Microspherocytes are intensely stained - less than 6.0 µm. Poikilocytosis is a change in the shape of red blood cells (sickle cell, target cell, ovalocytes, acanthocytes, stomatocytes, etc.) Anisochromia is a different color of red blood cells (hypo-, hyper-, normochromic, polychromasia) Sideroblasts are bone marrow erythrokaryocytes containing iron (normally 20-40 %)
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According to WHO recommendations: The lower limit of HB content in men is 130 g/l, in women – 120 g/l, in pregnant women – 110 g/l. The lower limit of erythrocyte content in men is 4.0 * 1012 / l, in women - 3.9 * 1012 / l. Hematocrit is the ratio of blood cells and plasma volume. Normally, in men it is 0.4-0.48%, in women it is 0.36-0.42%. Hb content in an erythrocyte: Hb (g/l) : Er (l) = 27-33 pg. Color index: Hb(g/l)*0.03: Er(l) = 0.85-1.0. Serum iron in men is 13-30 µmol/l, in women – 11.5-25 µmol/l.
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According to WHO recommendations: Total iron-binding capacity of blood serum (TIBC) is the amount of iron that can bind one liter of blood serum. Normally – 50-84 µmol/l, CVSS – syv. iron = latent FSSCC. Normal is 46-54 µmol/l. Syv. iron: TISS = transferrin saturation with iron. Normal is 16-50%. Assessment of iron reserves in the body: determination of ferritin in blood serum (radioimmune and enzymoimmune methods), normally – 12-150 µg/l, in men ≈ 94 µg/l, in women ≈ 34 µg/l; determination of protoporphyrin content in erythrocytes – 18-90 µmol/l; desferal test (desferal binds only iron reserves). 500 mg of desferal is administered intramuscularly; normally, 0.6-1.3 mg of iron is excreted in the urine.
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Etiopathogenetic classification of anemia Etiopathogenetic classification of anemia Acute posthemorrhagic (APHA) Iron deficiency (IDA) Associated with impaired synthesis or utilization of porphyrins (sideroachrestic) (SAA) Associated with impaired DNA and RNA synthesis (B12 and folate deficiency, megaloblastic) (MGBA) Hemolytic (HA) Aplastic , hypoplastic - with inhibition of bone marrow cells (AA) Other types of anemia: in infectious diseases, kidney diseases, liver diseases, endocrine pathologies, etc. Classification of anemia by pathogenesis Anemia due to blood loss (OPHA, IDA) Anemia due to impaired blood formation (IDA, CAA, MGBA, AA) Anemia due to increased blood destruction (HA)
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Classification of anemia by color index Classification of anemia by color index Hypochromic (IDA, SAA, thalassemia) Hyperchromic (MGBA) Normochromic (OPHA, AA, GA) According to the state of bone marrow hematopoiesis Regenerative (IDA, MGBA, SAA, OPGA) Hyperregenerative (HA) Aregenerative ( AA) Reticulocyte - the youngest cell of the erythroid series, which extends to the periphery - this is an indicator of germ regeneration (normal 1.2 - 2%) By severity Mild (Hb 110-90 g/l) Moderate (Hb 90-70 g\l) l) Heavy (Hb 70-50 g/l)
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Stages of diagnosis for anemia syndrome History, to identify the possible cause of anemia (heredity, provoking factors). Examination, determination of anemia variant. Mandatory research methods: CBC (Er, Hb, CP or Hb content in Er) Ht (hematocrit) reticulocytes (N = 1.2-2%) leukocytes and platelets serum iron sternal puncture with bone marrow examination (cellular composition, ratio of cells in bone marrow)
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Stages of diagnosis for anemia syndrome Additional research methods: trepanobiopsy of the ilium (tissue relationship in the bone marrow: cells/fat = 1/1) Coombs test urine for hemosiderin osmotic resistance of erythrocytes hemoglobin electropharesis life expectancy study Er c Cr51. Determination of the underlying disease that led to anemia: feces for occult blood (Gregersen or Weber methods). Calculation of fecal radioactivity within 7 days after intravenous administration of own washed erythrocytes labeled with Cr51. Study of radioactive iron given orally, followed by determination of radioactivity in stool over several days (normally 20% of iron is absorbed); EGDFS; RRS, irrigo-, colonoscopy; consultation of women with a gynecologist; study of the blood coagulation system, etc.
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Vitamin B12 and folic acid take part in the main stages of the exchange of purine and pyrimidine bases during the synthesis of DNA and RNA. Vitamin B12 and folic acid take part in the main stages of the exchange of purine and pyrimidine bases during the synthesis of DNA and RNA. The body contains 4 mg of vitamin B12, which is enough for 4 years.
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Causes of vitamin B12 deficiency Insufficient B12 content in food. Malabsorption: violation of gastromucoprotein synthesis: atrophic gastritis of the fundus of the stomach; autoimmune reactions with the production of antibodies to gastric parietal cells and gastromucoprotein; gastrectomy (after gastrectomy, the half-life of B12 is 1 year; after gastrectomy, signs of B12 deficiency appear after 5-7 years); stomach cancer; congenital deficiency of gastromucoproteins; impaired absorption of B12 in the small intestine; diseases of the small intestine accompanied by malabsorption syndrome (chronic enteritis, celiac disease, sprue, Crohn's disease) resection of the ileum; small intestine cancer; congenital absence of receptors for the vitamin B12 + gastromucoprotein complex in the small intestine; competitive uptake of vitamin B12; broad tapeworm infestation; pronounced intestinal dysbiosis. Reduced production of transcobalamin-2 in the liver and impaired transport of vitamin B12 to the bone marrow (in liver cirrhosis).
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The main differential criteria for B12-deficiency anemia Circulatory-hypoxic syndrome No sideropenic syndrome Gastroenterological syndrome: decreased appetite, body weight, glossitis (smooth red tongue), heaviness in the epigastrium, unstable stool, achlorhydria, m.b. hepatosplenomegaly Neurological syndrome (funicular myelosis): dystrophic processes in the posterolateral columns of the spinal cord associated with the accumulation of toxic methylmalonic acid, manifested by: impaired sensitivity of the limbs, changes in gait and coordination of movements, stiffness of the lower extremities, impaired movements of the fingers, ataxia, impaired vibration sensitivity.
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Hematological syndrome: Hematological syndrome: hyperchromic anemia (CP above 1.1-1.3); anisocytosis (megalocytosis), poikilocytosis, basophilic granularity, Cabot rings, Jolly bodies; trilinear cytopenia; hypersegmental neutrophilosis; megaloblastic type of hematopoiesis (according to sternal puncture); decrease in B12 in the blood is less than 200 pg/ml;
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It is less common than B12-deficient. It is less common than B12-deficient. The reserve of FA in the body is designed for 2-3 months. FA is present in all products; when heated, it is destroyed. Absorbed in the entire jejunum, maybe. diarrhea Transport proteins are not needed for the absorption of FA Congenital defects of FA are combined with mental retardation and are not corrected by the introduction of FA
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Main differential criteria for folate-deficiency anemia History data: pregnancy, neonatal period, chronic alcoholism, chronic hemolysis, myeloproliferative diseases, medication (folic acid antagonists, antituberculosis, anticonvulsants). Erythropoiesis suffers. There is no funicular myelosis or gastric damage. There is no reticulocyte crisis when taking B12. In the bone marrow, megaloblasts are stained with a dye only in B12-deficiency anemia, but not in folate-deficiency anemia. Reduction of folic acid in the blood is less than 3 mg/ml (N – 3-25 mg/ml).
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Treatment of megaloblastic anemia (MGBA) Vitamin B12 (cyanocobalamin) - 400-500 mcg intramuscularly (4-6 weeks). For neurological disorders: B12 (1000 mcg) + cobalamide (500 mcg) until neurological symptoms disappear. If necessary, lifelong administration of B12 (500 mcg) once every 2 weeks or preventive treatment - B12 (400 mcg) for 10-15 days 1-2 times a year. Erythromass transfusion only for health reasons (for all anemias!): Nb< 50 г/л, Нв < 70 г/л с нарушением гемодинамики, развитие прекомы и комы, срочная подготовка к операции и т.д. Дегельминтизация – выведение лентеца широкого (феносал, мужской папоротник). Фолиевая кислота 5-15 мг/сут (до 30 мг/сут); профилактическая доза – 1-5 мг/сут. Критерии эффективности лечения субъективные улучшения в первые дни лечения; ретикулоцитарный криз на 5-7 день лечения; улучшение показателей крови ко второй неделе лечения, с нормализацией через 3-4 недели.
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Aplastic anemia (AA) AA is a hematological syndrome caused by a large number of endogenous and exogenous factors, qualitative and quantitative changes in the stem cell and its microenvironment, the cardinal morphological feature of which is pancytopenia in the peripheral blood and fatty degeneration of the bone marrow. P. Ehrlich (1888) first described AA. The term “aplastic anemia” was introduced in 1904 by Shoffar. Incidence 4-5 people per 1 million population per year (in Europe) Age peaks of incidence 20 and 65 years
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Etiological factors of AA Etiological factors of AA drugs, chemicals, viruses, autoimmune processes; in 50% of cases the etiology is unknown (idiopathic AA). Pathogenesis of AA Functional bone marrow failure with inhibition of 1, 2 or 3 germs (pancytopenia). Damage to pluripotent blood stem cells Suppression of hematopoiesis Action of immune (cellular, humoral) mechanisms Deficiency of factors that stimulate hematopoiesis Iron, B12, protoporphyrin cannot be used by hematopoietic tissue.
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Aplastic anemia can be Aplastic anemia can be Congenital (with or without congenital anomaly syndrome) Acquired Along the course, AA Acute Subacute Chronic Forms of AA Immune Non-immune Clinical syndromes AA Circulatory-hypoxic Septic-necrotic Hemorrhagic
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Data from laboratory and instrumental studies Data from laboratory and instrumental studies of CP and iron content in erythrocytes are normal (normochromic A), reticulocytes are reduced (aregenerator A), increased serum iron, transferrin saturation with iron by 100%, erythrocytes ↓, NV ↓ (up to 20- 30 g/l), thrombocytopenia (m.b. up to 0), leukopenia (m.b. up to 200 in μl), liver, spleen and lymph nodes are usually not enlarged, bone marrow (trepanobiopsy of the ilium): aplasia of all sprouts, replacement bone marrow fatty. In 80% of AA - pancytopenia, 8-10% - anemia, 7-8% - anemia and leukopenia, 3-5% - thrombocytopenia.
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Severe AA Severe AA In peripheral blood (2 out of 3 germs are suppressed) Granulocytes 0.5-0.2*109/l Platelets less than 20*109/l Reticulocytes less than 1% Myelogram Myelokaryocytes less than 25% of the norm Myelokaryocytes 25-50%, and myeloid cells less than 30% Trephine biopsy When mild form– 40% of adipose tissue In moderate – 80% In severe – absolute predominance of adipose tissue (panmyelophthisis) Differential diagnosis AA Debut of acute leukemia Chronic lymphocytic leukemia (bone marrow form) Cancer metastases to the bone marrow Pancytopenia in older people, as a manifestation of B12-deficiency anemia
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Treatment of AA Bone marrow restoration: cyclosplrin A (sandimmune), antilymphocyte Ig (ALG), antiplatelet Ig (ATG), corticosteroids, donor bone marrow transplantation (performed in severe cases at age<40 лет, в ранние сроки). Заместительная терапия компонентами крови. Асептические условия; купирование и профилактика инфекции (АБТ). Если АТ, то плазмоферез. Андрогенные стероиды (нерабол, ретаболил). Спленэктомия. Колониестимулирующие факторы (агранулоцитарный колониестимулирующий фактор – лейкомакс; гранулоцитарный колониестимулирующий фактор - лейкоген). Эритропоэтин, тромбопоэтин. При необходимости выведение избытков железа.
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Evaluation of AA therapy Complete remission: HB > 100 g/l; granulocytes > 1.5*109/l; platelets > 100*109/l; no need for blood transfusions. Partial remission: HB > 80 g/l; granulocytes > 0.5*109/l; platelets > 20*109/l; no need for blood transfusions. Clinical and hematological improvements: improvement of hematological parameters; reduced need for blood replacement transfusion for more than two months. Lack of effect: no hematological improvements; the need for blood transfusion remains.
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Systems whose disruption causes hemolysis Glutathione system: protects important cell components from denaturation by oxidizing agents, peroxides, ions heavy metals. Phospholipids: determine the permeability of the membrane for ions, determine the structure of the membrane, influence the enzymatic activity of proteins. Red blood cell membrane protein: 20% spectrin – a heterogeneous mixture of polypeptide chains; 30% – actomyosin. Glycolysis is a method of anaerobic conversion of glucose into lactic acid, during which ATP is formed - the accumulator of chemical energy of cells. Other substrates of glycolysis: fructose, mannose, galactose, glycogen. The pentose phosphate cycle is an anaerobic oxidative pathway for the conversion of glucose. Adenyl system: adenylate kinase and ATPase.
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Hemolytic anemia (HA) HA combines a number of hereditary and acquired diseases, the main symptom of which is increased decay of Er and a shortening of their life expectancy from 90-120 to 12-14 days. Hereditary HA are associated with defects in the Er structure, which become functionally defective. Acquired HAs are caused by various factors that contribute to the destruction of Er (hemolytic poisons, mechanical effects, autoimmune processes, etc.). Pathological hemolysis can be 1. By localization intracellular (RES cells, mainly the spleen) intravascular 2. By course acute chronic
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Main criteria for GA Increased bilirubin due to unconjugated bilirubin: bile pigments in the urine are negative; urobilin in urine and stercobilin in feces; “lemon” jaundice without itching. Splenomegaly with intracellular hemolysis. Anemia: normochromic, hyperregenerative, hyperplasia of the erythroid lineage in the bone marrow. Hemolytic crises. M.b. gallstones (pigment stones). Intravascular hemolysis is characterized by: hemoglobinemia (free HB in the blood plasma); hemoglobinuria and hemosiderinuria (red or black urine); hemosiderosis internal organs; tendency to microthrombosis of various localizations.
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HA with intravascular hemolysis Hereditary HA: A. Enzymopathies (G-6-PD deficiency). B. Hemoglobinopathies (sickle cell anemia). 2. Acquired HAs: A. Immune - AIHA with thermal and biphasic hemolysins. B. Non-immune – PNH, mechanical for prosthetics of valves, vessels, marching.
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Classification of hereditary hemolytic anemias A. Membranopathy due to disruption of the structure of the erythrocyte membrane protein Microspherocytosis, elliptocytosis, stomatocytosis, piropoikilocytosis. Disorders of erythrocyte membrane lipids: acanthocytosis, deficiency of lecithin-cholesterol-aryltransferase activity, increased lecithin content in the erythrocyte membrane, infantile pycnocytosis.
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B. Enzymopathies B. Enzymopathies Deficiency of enzymes of the pentose phosphate cycle. Deficiency of the activity of glycolysis enzymes. Deficiency of the activity of glutathione metabolism enzymes. Deficiency in the activity of enzymes involved in using ATP. Deficiency of ribophosphate pyrophosphate kinase activity. Impaired activity of enzymes involved in the synthesis of porphyrins. B. Hemoglobinopathies Caused by an anomaly in the primary structure of Hb. Caused by a decrease in the synthesis of polypeptide chains that make up normal Hb. Caused by a double heterozygous state. Anomalies of HB not accompanied by the development of the disease.
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Hereditary enzymopathies Insufficiency of glucose-6-phosphate dehydrogenase (G-6-FDG) in Er It is more common in the countries of Africa, Latin America, the Mediterranean, in our country - Azerbaijan, Armenia, Dagestan; Suffer mainly from men (recessive sex-linked gene); Provoke a crisis acute infections, medications(paracetamol, nitrofurans, sulfonamides, tuberculostatics, etc.) and some legumes, acidosis in diabetes and chronic renal failure. Intravascular hemolysis. The morphology of Er is not changed. Osmotic resistance Er in N or slightly. After a crisis in Er, Heinz bodies (denatured Hb) can be detected. Diagnosis in the group of hereditary fermentopathy is based on the detection in Er of a deficiency of various enzymes of the hexose or pentose cycles.
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Membranopathies The most common among them is hereditary microspherocytosis (Minkowski-Choffard disease), in which a defect in the Er membrane is accompanied by an increase in the passage of Na and H2O ions into the cell with the formation of a spherocyte. The spherocyte, passing through the sinuses of the spleen, decreases in diameter from 7.2-7.5 microns to< 6 (при этом кривая Прайс-Джонса сдвигается влево). Внутриклеточный гемолиз. Гемолитические кризы провоцируются инфекциями, переохлаждением, беременностью и др. Характерно снижение осмотической резистентности Эр: min до 0,6-0,7, max до 0,4% (в N – min – 0,46-0,48, max – 0,32-0,34%). Прибавление к Эр глюкозы значительно уменьшает гемолиз.
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Hemoglobinopathies Hereditary HA with impaired synthesis of the protein part of Hb. The Hb molecule consists of 4 heme molecules and 4 polypeptide chains (2 α and 2 β). Substitution of amino acids in polypeptide chains leads to the formation of pathological Hb (S, F, A2, etc.). The disease occurs more often in homozygotes in the Mediterranean countries, Africa, India and the Transcaucasian republics. Homozygous patients have severe, sometimes fatal manifestations of the disease from childhood, while heterozygotes have mild forms with survival > 20-30 years. Er's lifespan has been shortened. The site of hemolysis is examined using Cr51-labeled Er. Anomalies of HB (S, F, A2, etc.) are detected by HB electrophoresis (immunophoresis). Maybe quantitation abnormal Hb.
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Classification of acquired hemolytic anemias A. Immune hemolytic anemias HA associated with exposure to antibodies (immune HA): isoimmune (alloimmune): Rh conflict, transfusion of incompatible blood; heteroimmune, caused by diseases, viruses; transimmune - antibodies are transmitted through the placenta from mother to fetus; Autoimmune HAs with antibodies to their own unchanged Er: with incomplete warm agglutinins (detected in 70-80% of autoimmune HAs using a direct Coombs test), with warm hemolysins, with complete cold agglutinins, associated with biphasic cold hemolysins. Autoimmune HA with antibodies against bone marrow normocyte antigen.
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Acquired HA Paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli disease) A clone of defective Er is formed due to somatic mutation type benign tumor blood systems with 2 populations of Er: with normal and defective membrane; simultaneously leukocytes and platelets mutate with the development of pancytopenia; intravascular hemolysis; a change in blood pH towards acidosis in the presence of complement leads to hemolysis (Hem, Crosby, sucrose tests); direct Coombs test is negative.
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Treatment of autoimmune GA Glucocorticoid hormones in the acute phase with thermal agglutinins; prednisolone 60-80 mg/day, divided into 3 doses at a rate of 3: 2: 1. With chronic course HA with incomplete thermal agglutinins Prednisolone 20-40 mg/day. In HA with complete cold agglutinins, with severe exacerbation, prednisolone 20-25 mg/day. Splenectomy – in case of ineffectiveness of hormones, rapid relapses after hormone withdrawal, complications of hormone therapy. Cytostatics: azathioprine 100-150 mg/day; cyclophosphamide 400 mg every other day; vincristine 2 mg once a week intravenously; chlorobutine 2.5-5 mg/day for 2-3 months – in the absence of effect from hormones. Transfusion of washed red blood cells, selected according to indirect sample Coombs for severe anemia. Plasmapheresis for severe HA, complicated by DIC syndrome. Immunoglobulin C 0.5-1 g/kg body weight.
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Principles of treatment of HA with intravascular hemolysis Infusion therapy– prevention of acute renal failure: soda, glucose solution with inulin, aminophylline 10-20 ml, furosemide 40-60 mg, mannitol 1 g/kg body weight. Prevention of DIC - small doses of heparin. Fighting infection - antibiotics (sickle cell anemia). Increasing acute renal failure - peritoneal dialysis, hemodialysis.
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Treatment of hemolytic crisis Restitution of circulating blood volume: rheopolyglucin 400-800 ml; reoglumal 400-800 ml; isotonic solution sodium chloride 1000 ml; albumin 10% 150-200 ml under the control of central venous pressure. Neutralization of toxic products and stimulation of diuresis. Hemodez (low molecular weight polyvinylpyrrolidone, colloidal solution) 300-500 ml, 2-8 infusions per course. Polydesis 250-1000 ml. Stimulation of diuresis: furosemide 40-80 mg intravenously, if necessary, again after 4 hours. Eufillin solution 2.4% 10-20 ml per 10 ml of isotonic sodium chloride solution (in the absence of arterial hypotension).
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Elimination of acidosis: 4% 200-400 ml of sodium bicarbonate intravenously. Elimination of acidosis: 4% 200-400 ml of sodium bicarbonate intravenously. Extracorporeal therapy - in the absence of effect from the above measures - plasmapheresis, hemodialysis. Glucocorticoid hormones: for autoimmune HA, shock, collapse - prednisolone intravenously 1-1.5 mg/kg of the patient’s body weight, again after 3-4 hours (if necessary). Relief of anemia: when HB decreases to 40 g/l and below - transfusion of individually selected red blood cells of 150-300 ml; red blood cells should be washed 4-5 times, fresh frozen, selected using the indirect Coombs test. In case of crisis against the background of NPG, red blood cells are 7-9 days old from the moment of preparation (fresh ones increase the risk of hemolysis).
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Treatment of sickle cell anemia Prevention of dehydration Prevention infectious complications(from 3 months to 5 years - penicillin 125-250 mg orally daily; after 3 years - polyvalent vaccination pneumococcal vaccine). Transfusion of washed or thawed red blood cells is the main method of treatment in adults and children. Indications for red blood cell transfusion: severe anemia, decreased reticulocytes; stroke prevention; blood transfusions reduce the content of Hb6 in erythrocytes and reduce the risk of stroke; preparation for abdominal operations; trophic ulcers shins; taking folic acid 1 mg/day daily in the presence of anemia.
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Treatment of thalassemia Treatment of the homozygous form: transfusion of washed or thawed red blood cells to maintain the HB level within 90-100 g/l; when frequent blood transfusions are complicated by hemosiderosis - desferal (complexone that removes iron from the body) at a dose of 10 mg/kg body weight taken orally ascorbic acid 200-500 mg; in the presence of splenomegaly, hypersplenism - splenectomy Treatment of the heterozygous form: folic acid 0.005 2 times a day; Iron supplements are contraindicated.
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Treatment of paroxysmal nocturnal hemoglobinuria Transfusion of washed or freshly frozen red blood cells with a shelf life of at least 7 days in case of severe anemia; in the presence of anti-erythrocyte or anti-leukocyte antibodies - transfusion of red blood cells selected using an indirect Coombs test. Anabolic hormones: Nerobol 0.005*4 times a day for at least 2-3 months under the control of cholestasis indicators. Antioxidants: vitamin E – erevit intramuscularly 3-4 ml/day (0.15-0.2 g tocopherol acetate); in capsules of 0.2 ml of 5% vitamin E solution, 2 capsules per day after meals; course 1-3 months. In case of severe iron deficiency, use iron supplements in small doses (Ferroplex 1 tablet 3 times a day) under the control of bilirubin levels. Treatment of thrombosis: heparin 2.5 thousand 2 times a day under the skin of the abdomen.
Anemia criteria (WHO): for men: hemoglobin level 
Clinical and pathogenetic classification of anemia: I. Anemia caused by acute blood loss II. Anemia resulting from deficient erythropoiesis III. Anemia resulting from increased destruction of red blood cells. IV.* Anemia developing as a result of combined causes;
II. Anemia resulting from deficient erythropoiesis Due to impaired maturation (microcytic): Iron deficiency; Impaired iron transport; Impaired iron utilization; Impaired iron recycling; 2) Due to impaired differentiation of erythrocytes; A/hypoplastic anemia (congenital, acquired) Dyserythropoietic anemia; 3) Due to disruption of the proliferation of erythropoiesis precursor cells (macrocytic); B12 deficiency; Folate-deficient;
III. Anemia resulting from increased destruction of red blood cells 1) Acquired hemolysis (non-erythrocyte causes): Autoimmune; Non-immune (poisons, medications, etc.) Traumatic (artificial valves, hemodialysis); Clonal (CNG); 2) Hemolysis caused by erythrocyte abnormalities: Membranopathies; Enzymopathies; Hemoglobinopathies; 3) Hypersplenism – intracellular hemolysis (the platelet level decreases first, anemia develops later);
Description: Any infectious or inflammatory disease organism are accompanied by a decrease in the level of red blood cell production in the bone marrow, and this leads to their quantitative decrease in the blood. But, anemia in chronic diseases can only develop if the disease is chronic and severe. The level of anemia directly depends on the level of severity chronic disease.
So, anemia of chronic diseases occurs in the following cases: chronic infections, inflammatory chronic processes in the body, with chronic renal failure, with collagenosis, malignant tumors, for diseases endocrine system, chronic liver diseases and pregnancy. Chronic diseases most often lead to anemia of different nature in old age. And the most popular type of anemia then is iron reutilization anemia, when the body’s ability to absorb iron decreases, while the life span of red blood cells is shortened and microscopic blood loss occurs in the body.
Symptoms Anemia of chronic diseases, due to its slow development and occurrence in a mild form (accompanying), as a rule, does not have any symptoms. All manifestations usually relate to those diseases against the background of which, or as a result of which, anemia develops. And yet, the symptoms that manifest developing anemia include increased fatigue of the body, its general weakness, a sharp decline performance, obvious irritability, frequent dizziness, drowsiness, noise sensations in the ears, spots before the eyes, rapid heartbeat and shortness of breath when physical activity or at rest.
Diagnostics All methods used to diagnose anemia of chronic diseases depend on the chronic disease itself, against which the anemia develops. But, in any case, if anemia occurs in the body, then mandatory the patient is prescribed general and biochemical analysis blood and bone marrow puncture to determine the nature and type of anemia.
Treatment Anemia that develops against the background or as a result of a chronic disease does not require separate treatment. All methods in in this case will be aimed at eliminating the cause of anemia, that is, at treating the chronic disease itself. When diagnosing, primary anemia must be excluded, and then, for each specific case, a course of treatment and therapeutic technique is selected. For example, kidney inflammation are treated with replacement therapy with erythropoietin, which leads to the correction of developing anemia. To reduce the severity of the anemic process and improve general condition Erythropoietin can be administered to the patient subcutaneously in moderate dosages, followed by their subsequent reduction. This is done no more than three times every seven to eight days. When treating anemia with erythropoietin, strict medical supervision of intravenous and intracranial blood pressure patient, since this drug can cause stroke, thrombosis and hypertension. In rare exceptional cases, when anemia of a chronic disease takes severe form, a treatment method such as red blood cell transfusion is used. Methods can also be used hormone therapy and blood transfusions (hemotransfusion).
Primary prevention - carried out to a group of people who do not have this moment anemia, but there are predisposing circumstances: pregnant and lactating women. All pregnant women with a gestational age of 8 weeks are divided into groups: 0 (zero) – normal pregnancy. Assign prophylactic appointment Fe (30-40 mg) from 31 weeks of pregnancy for 8 weeks. Group 1 – pregnant women with normal analysis blood, but with risk factors (gastrointestinal pathology, heavy and prolonged menstruation before pregnancy, multiple births, insufficient intake of Fe from food, the presence of infection, early toxicosis with frequent vomiting). Preventive therapy begins from the 12-13th week to the 15th, then from the 21st from the 31st to the 37th week. Group 2 – women who developed anemia during pregnancy. Therapeutic doses of drugs are used. Group 3 – women with pregnancy that occurred against the background of pre-existing IDA. Treatment is carried out with the appointment therapeutic doses drugs, then saturation therapy and courses preventive therapy(2 courses of 8 weeks) in combination with taking antioxidants (Vit E, Aevita, Vit C, multivitamins, calcium supplements) Teenage girls and women with heavy and prolonged periods (prescribe 2 courses of preventive therapy for 6 weeks or after menstruation for 7-10 days throughout the year.
