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Hemolytic anemia class 31 december.pptx

  1. HEREDITARY SPHEROCYTOSIS • Inherited disorder • Autosomal dominant • Incidence 1: 5000 (underestimate) • Pathogenesis -Molecular abnormality -Involves spectrin & ankyrin
  2. Spherocytes
  3. Major clinical features • Anaemia • Jaundice • Splenomegaly • Severity of hemolysis varies & so clinical features • Mostly – asymptomatic • Compensated chronic hemolytic state • Few – severe hemolysis
  4. Crisis • Hemolytic – associated infection • Megaloblastic – pregnancy associated • Aplastic – parvovirus B19
  5. Associated symptoms • Pigment gallstones – symptomatic in 50% • Chronic leg ulcers
  6. HEREDITARY ELLIPTOCYTOSIS • AR or AD • 1 per 4000 to 5000 • Red cells of oval or elliptic shape are normally found in birds, reptiles & camels. • In humans – in Hereditary Elliptocytosis
  7. HEREDITARY ELLIPTOCYTOSIS • Elliptic shape is acquired as the cell deforms to traverse the micro circulation but does not spring back to its initial biconcave shape. • Abnormality in anchor protein – α spectrin or protein 4.1. • Offers relative protection from malaria.
  8. Elliptocytes
  9. RED CELL ENZYMOPATHIES • During its maturation, RBC loses its nucleus, ribosomes & mitochondria –> loses its capacity for protein synthesis & oxidative phosphorylation. • Mature RBC has relatively simple pattern of intermediary metabolism.
  10. • ATP generated from Embden – Meyerhoff Pathway –> drives the cation pump –> ionic milieu within the RBC Pyruvate kinase Hexokinase • HMP shunt – protects from oxidative stress Those with deficiency of HMP enzyme – unable to maintain an adequate level of reduced glutathione in their RBC Hb sulphydryl groups become oxidized –> Hb tends to precipitate within RBC forming Heinz bodies
  11. G-6- P D DEFECIENCY • Most common enzymopathy • Affects 10% of world population • G6PD gene located on X-chromosome • Sex linked trait • 400 subtypes of G6PD – B common
  12. Clinical features: • Variable • Acute drug induced hemolysis a) Analgesics -Aspirin, phenacetin b)Antimalarials c)Antibiotics-Sulphonamides,Ciprofloxacin d)Misc – Dapsone,Quinidine
  13. Chronic compensated hemolysis Infection or acute illness Neonatal jaundice Favism – broad beans
  14. DIAGNOSIS • During attack: nonspherocytic intravascular hemolysis
  15. DIAGNOSIS • Peripheral blood smear -bite cells -blister cells -irregular shaped small cells -Heinze bodies
  16. CELL DESCRIPTION • Bite cells are red blood cells that contain a semi-circular indent on the edge of their membrane, giving the appearance of a bite being taken out of the cell. • Blister cells on the other hand, have cytoplasmic projections that fuse together, creating a vacuole on the edge of the membrane, giving the appearance of a blister.
  17. Blister cell
  18. CELL FORMATION • Bite and Blister cells are often seen together, and may form through various mechanisms Red blood cells originally containing inclusions are “pitted” or removed by macrophages in the spleen, resulting in bite or blister cells When the red blood cell is impaled by fibrin strands, the membrane can reform and produce a vacuole which results in a blister cell Bite cells can also form when a blister cell ruptures
  19. DIAGNOSIS • G 6 P D levels Screening - if low then Direct quantitative assessment
  20. TREATMENT • Stop offending drug • Treat underlying infection • Acute hemolysis- blood transfusion
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