anaemia. definition decreased haemoglobin concentration a decrease in normal number of red blood...

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