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Red Blood Disorders Anemia Lviv National Medical University Department of pathological physiology PATHOLOGY OF RED BLOOD PhD. Sementsiv N.G. In norm The number of erythrocytes: in female - 3,9-4,7·1012/l in male - 4,5-5,0·1012/l Hemoglobin in female - 120-140g/l in male - 140-160g/l Color index(CI) - 0,85-1,15 Globular value = 3 x Hb / the first 3 figures of erythrocytes. Reticulocytes - 0.5-2%, 0,5-2%0 changes in total blood volume normovolemia hypovolemia hypervolemia simple (Ht - norm), polycitemia (Ht > 0,48), olygocytemia (Ht < 0,36). A) Norm B) acut anaemia б) acute hemorrhage г) hydremia Pathological forms of erythrocytes regenerative degenerative cell pathologic regeneration Regenerative forms reticulocytes залежно від зрілості розрізняють: (Зернисті) Stippling (Сітчасті) Mesh Norm in a blood reticulocytes - 0,2–2,0%. Regenerative forms Basophiles substantial erythrocytes - cytoplasm remains basophilic normo blast. Polychromatophil erythrocytes (polychromasia, polychromatophilia ) – erythrocytes with basophiles substantial ( blue cells) indicates increased RBC production by the marrow Qualitative (degenerative) changes of red blood cell - poikilocytosis - different shape of erythrocytes; - anisocytosis - different size of erythrocytes; - anisochromia - different saturation of red blood cells by hemoglobin Degenerative forms Anisocytosis present in a blood different forms erythrocytes » normocyte (7,01–8,0 мкм) » microcyte(6,9–5,7 мкм) » macrocyte(8,1–9,35 мкм) » megalocyte (10–15 мкм) Degenerative forms Poikilocytosis present in a blood pictures erythrocytes different forms : elongate form , oval, ellipsoid and os. ОVAlOCYTE( ELLIPTOCYTE) – 5% all blood. Pathological cells regenerate Megaloblast mehaloblastyc cell type hematopoiesis Megaloblast oval cells in the diameter of 1,5- 2,0 times larger than normal erythrocytes is the final stage mehaloblastyc hematopoiesis. - pathological inclusions in the erythrocytes : Cabot rings Jolly bodies Cabot rings Jolly bodies Erythrocytosis Primary family (hereditary) forms Secondary Absolute (increased erythropoiesis) relative Vaquez disease Erythremia (true polycythemia) - chronic leukemia with the defect of cells of precursor of mielopoesis with unlimited proliferation hematopoetic cells. Blood picture: Hemoglobin (over 180g/l) Erythrocyte (over 6x1012/l) Platelets (over 400x109/l) Leukocyte (over 10x109/l) Hematocrit ↑ Anaemia Anemia -is a syndrome or disease which is characterized by the decrease of erythrocytes and hemoglobin amount in the unite of blood volume and accompanied by qualitative changes of erythrocyte. Cases of anemia The cases of anemia are numerous but the most important grouped whit in three major mechanisms that produce anemia: Blood loss (excessive bleeding) Inadequate production of red blood cells Excessive destruction of red blood cells Classification of anemia I. According to etiology: Hereditary and acquired II. According to pathogenesis : Diserythropoietic Hemolytic Posthemorragic IІІ. According to type of hematopoesis: 1. with erythroblastic 2. with megaloblastic ІV. According to color index (globular value): 1. normochromic 2. hypochromic 3. hyperchromic Color index(CI) - 0,85-1,15 Globular value = 3 x Hb (g/l) / the first 3 figures of erythrocytes. ІV. According to color index (globular value): Hypohromatic (c.i. < 0,8) Normochromatic (c.i. = 0,8– 1,05) Hyperchromatic (c.i. > 1,05) V. According to ability of bone marrow to regeneration: regenerative hyperregenerative hyporegenerative aregenerative Classification of anemia Over a mean diameter of erythrocytes (MDE) normocytic (mde = 7,2–8,0 мкм) microcytic (mde < 7,2 мкм) macrocytic (mde = 8,1–9,5мкм) megalocytic anaemia (mde > 12 мкм) Classification of anemia By type hematopoiesis Megaloblastic Erythroblastic Classification of anemia By etiopathogenesis Posthemorrhagic Hemolityc Dyserythropoietyc Classification of Anemia According to Underlying Mechanism I Blood Loss Acute: trauma Chronic: lesions of gastrointestinal tract, gynecologic disturbances II Increased Destruction (Hemolytic Anemias): III Impaired Red Cell Production Classification of Anemia According to Underlying Mechanism II Increased Destruction (Hemolytic Anemias): A. Intrinsic (intracorpuscular) abnormalities 1. Hereditary 2. Acquired B. Extrinsic (extracorpuscular) abnormalities Classification of Anemia According to Underlying Mechanism B. Extrinsic (extracorpuscular) abnormalities 1. Antibody mediated 2. Mechanical trauma to red cells: Extrinsic (extracorpuscular) abnormalities 1. Antibody mediated a. Isohemagglutinins: transfusion reactions, erythroblastosis fetalis (Rh disease of the newborn) b. Autoantibodies: idiopathic (primary), drug-associated, systemic lupus erythematosus B. Extrinsic (extracorpuscular) abnormalities 2. Mechanical trauma to red cells: a. Microangiopathic hemolytic anemias: thrombotic thrombocytopenic purpura, disseminated intravascular coagulation b. Infections: malaria Toxic hemolytic anemia Poisoning by hemolytic poisons: Arsine Acetic lead Phosphorus Nitrobenzene Phenylhydrazine Biliary acids Mushroom poison Snake venom Immune hemolytic anemia 1. Autoimmune anemia 2. Post transfusion anemia а) transfusion of АВ0-uncompatible blood б) transfusion of Rh- uncompatible blood 3. Hemolytic anemia of new-born Acquired haemolytic anemia Ethiology Immune injury : – isoimmun; – autoimmun. Injury caused by biological factors: the endo-and Some strains of microbes. Mechanical injury Thermal injury . Intoxication hemolytic poisons . A. Intrinsic (intracorpuscular) abnormalities 1. Hereditary a. Membrane abnormlities Membrane skeleton proteins: spherocytosis, elliptocytosis: Membrane lipids: abetalipoproteinemia b. Enzyme deficiencies Glycolytic enzymes: pyruvate kinase, hexokinase Enzymes of hexose monophosphate shunt: glucose-6-phosphate dehydrogenase, glutathione synthetase Microspherocytosis or Minkowsky-Shauffard disease [hereditary spherocytosis]. Membranopathy Blood picture : 1. The number of hemoglobin decreases. 2. The number of erythrocytes is decreases. 3. CI is normal - normochromic anemia. 4. The reticulocytes number increases - hyperregenerative anemia. 5. Decrease of erythrocytes osmotic resistance takes place. 6. Increase mean concentration of hemoglobin in the red blood cell (МСНС) more than 36%. 6. The number of unconjugated [indirect] bilirubin increases. Enzymopathy-deficiency of the enzyme Gl-6-FDG Blood picture : 1. Erythrocyte number ↓ 2. Hemoglobin amount ↓ 3. Color index (globular value) – normochromic. 4. Reticulocyte number ↑ 5. Erythrocytes osmotic resistance ↓ 6. Indirect bilirubin ↑ A. Intrinsic (intracorpuscular) abnormalities 1. Hereditary c. Disorders of hemoglobin synthesis Deficient globin synthesis: thalassemia syndromes Structurally abnormal globin synthesis (hemoglobinopathies): sickle cell anemia, unstable hemoglobins: Hemoglobinopathys are the anemias related to the violation of hemoglobin synthesis. Types of hemoglobinopathys : The 1st – is the hemoglobin chains synthesis violations; The 2nd – is the hemoglobin chains structure violations. Hemoglobinopathy α-thalassemia Blood picture : 1. Erythrocyte number ↓ ↓ 2. Hemoglobin amount ↓ 3. Color index ↓. 4. Reticulocyte number ↑ 5. Erythrocytes osmotic resistance ↓ 6. Indirect bilirubin ↑ 7. Target erythrocytes (codocytes) Sickle-cell anemia Blood picture : Erythrocyte number ↓ Hemoglobin amount ↓ Color index (globular value) – normochromic Indirect bilirubin ↑ Smear - Sickle-cell III Impaired Red Cell Production A.Disturbance of proliferation and differentiation of stem cells: 2. Defective hemoglobin synthesis a. Deficient heme synthesis: iron deficiency b. Deficient globin synthesis: thalassemias: Anemia of renal failure III Impaired Red Cell Production B. Unknown or multiple mechanisms: myelodysplastic syndrome, anemia of chronic inflammation, anemias due to marrow infiltrations Pathology of Anemias CLINICAL MANIFESTATIONS Acute: shortness of breath, organ failure, shock Chronic: With hemolysis: skeletal abnormalities because of expansion of marrow; growth retardation; jaundice and gallstones With defective erythropoiesis: iron overload, heart and endocrine failure Signs and symptoms of anaemia Most commonly, people with anemia report non-specific symptoms of a feeling of weakness, or fatigue, general malaise and sometimes poor concentration. Posthemorrhagic anaemia 1. Acute 2. Chronic Periods of compensation of acute blood loss 1. Reflectory 2. Hidremic 3. Medullary Iron-deficiency anemia Blood picture : Erythrocyte number ↓ Reticulocyte number ↓ Hemoglobin amount ↓ Color index (globular value) ↓ Smear: microcytes, anulocytes , aniso/poikilocytosis, Target and “pencil” cells serum ferritin ↓ serum iron ↓ Serum soluble transferine receptors increase State of the oral cavity in anemias • Dietary iron deficiency anemia or anemia of chronic disease should be suspected in every case of glossitis, glossodynia, angular cheilitis, erythematous mucositis, oral candidiasis, recurrent oral ulcers, and burning mouth when no other obvious causes are identified Glossodynia Iron Deficiency Anemia IRON REFRACTORY is anemia which arises up as a result of the violation of iron including into hemoglobin, at the activity decrease or absence of enzymes which are needed for the hemoglobin synthesis (protoporphyrin ІX deficiency) Reasons: 1. Hereditary 2. Lead poisoning, alcoholism. Iron-refractory anemia Blood picture :
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