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)– 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 heam synthesis)
– 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 globin synthesis)
• 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 -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 anaemiaCobalamin 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