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Anaemia. Definition. decreased haemoglobin concentration a decrease in normal number of red blood cells decreased haematocrit. Classification. Mechanism decreased production of Ery increased destruction or loss of Ery combined Size of red blood cells (MCV)
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Definition • decreased haemoglobin concentration • a decrease in normal number of red blood cells • decreased haematocrit
Classification • Mechanism • decreased production of Ery • increased destruction or loss of Ery • combined • Size of red blood cells (MCV) • microcytic – i.e. iron deficiency anaemia • normocytic – i.e. haemolytic anaemia • macrocytic - megaloblastic anaemia • Hb concentration in Ery (MCH) • hypochromic • normochromic • hyperchromic
Classification according to mechanism • Decreased production of Ery • Iron deficiency anaemia( synthesis of haem) • Sideroblastic anaemia(defect of heamsynthesis) • Megaloblastic anaemia( synthesis of DNA) • Pernicious anaemia • Aplastic anaemia(defect of erythropoesis) • Anaemia of chronic diseases - inflammatory, kidney, endocrinology, liver (inhibition of erythropoesis) • Sicle cell anaemia, talasemia(defect of globinsynthesis)
Increased destruction or loss of Ery • Posthemoragic anaemia • chronic bleeding (menstruation, gastric ulcers...) • Hemolytic anaemia • Congenital • Genetic conditions of RBC Membrane • Hereditary spherocytosis • Hereditary elliptocytosis • Genetic RBC enzyme defects • G6PD deficiency • Pyruvate kinase deficiency • Haemoglobinopathies • Sicle cell anaemia • Thalasemia
Spherocytes Eliptocytes Sicle cells
Glucose-6-phosphate dehydrogenase deficiencyFavism • Cause • gene mutation (X linked) • Manifestation after: • foods (beans) • drugs • infection • Effect • oxidative stress haemolysis
Hemolytic anaemia • Acquired • Immune mediated hemolytic anemia • Autoimmune hemolytic anemia • Warm (Cold) antibody autoimmune hemolytic anemia • Rh diesease • ABO hemolytic disease in newborn • Blood tranfusion reactions • Non-immune mediated hemolytic anaemia • Toxins (snake venom) • Trauma • Mechanical (heart valeves defects) • Infections • Membrane disorders (Paroxysmal nocturnal heamoglobinuria)
Combined • Talasemia • Sicle cell anaemia
Diagnosis • haemoglobin men 130 - 175 g/l women 120 - 165 g/l • haematocrit men 0,40 - 0,54 women 0,35 - 0,45 • RBC count men 4,2 - 5,8 x 1012/l women 3,8 - 5,2 x 1012/l • Mean corpuscular volume (MCV) MCV = Htk/RBC count norm - 80- 95 fl < 80 fl - microcytosis (i.e. iron def. anaemia) > 95 fl – macrocytosis (i.e. megaloblastic anaemia) • Mean corpuscular Hb (MCH) MCH = Hb/RBC count norm 27 - 32 pg < 27 pg - hypochromia > 32 pg - hyperchromia • Mean corpuscular Hb concentration (MCHC) MCHC = Hb/Htk norm 320 - 370 g/l < 320 gl - hypochromia Other tests: Erythrocyte sedimentation rate, ferritin, serum iron, total iron binding capacity, transferrin, folate level, serum vitamin B12...)
Signs and symptoms • decreased oxygen tranportation • dyspnea, fatique, lethargy, pale skin, mucosa, nail beds, angina pectoris (in patients with preexisting heart disease) • decreased plasma volume • postural hypotension • increased heart output • palpitations, murmurs, tachycardia • Specific signs • jaundice – haemolytic anaemia • koilonychia – iron deficiency • pica (the consumption of non-food based items such as dirt, paper, wax, grass...) - iron deficiency
koilonychia clubbed fingernails
Iron deficiency anaemia – sideropenic anaemia Causes • dietary insuficiency (vegetarians) • malabsorption • chronic blood loss • loss of blood in the stool... Symptoms • hypochromic, microcytic anaemia • fatique, pale skin, tachycardia • poor apetite, constipation, • hair loss, nail deformities • gingivitis, glossitis, gastritis
Sideroblastic anaemia • hypochromic anaemia • disorder of heme synthesis • hereditary • d-amino-levulinic acid synthetase deficiency • acquired • lead intoxication • ethanol • inflammation • chemotherapy sideroblastic anaemia
Megaloblastic anaemia • macrocytic anaemia • Disorder of DNA synthesis • deficiency of B12 vitamine • deficiency of folic acid • after some drug application • enzymopathies – rare Symptoms • general • GIT - glositis, anorexia, diarrhea • neurological – disorders of sensitivity, parestesia, ataxia
Megaloblastic anaemia Cobalamin deficiency • inadequate intake (vegetarians) • malabsorption • deficient intrinsic factor (athrophic gastritis) • Pernicious anaemia • resorption disorders (after gastric surgery) • biological competition • bacteria, parasites Folate deficiency • inadequate intake • alcoholism • malabsorption • increased need (pregnancy, infancy)
Aplastic anaemia • inadequate red cell production (or all blood cells – pancytopenia) in bone marrow Cause • idiopathic • autoimmune disorder • drugs • ionizing radiation Symptoms • general • thrombocytopenia – bruising, petechiae • leukopenia – risk of infection
Oral signs of anemia Oral leukoplakia in aplastic anemia Cheilosis