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Haemolytic Anaemia (Intravascular Haemolysis (haemopexin binds Hb after…

- - - - Ankyrin deficiency or abnormalities
      - Spectrin deficiency or abnormalities
      - Band 3 abnormalities
      - Palladin (protein 4.2) abnormalities
  - - - Spherocytes not able to pass through the
      - microcirculation of the spleen.
      - They remain there and die prematurely.
  - - - 5-20% reticulocyte
        
        Osmotic fragility test, right shift indicates SA:VOL ratio change
- - - - sugars are attached (glucuronides) and moves to gut
- - - - Oxidants
        
        Dapsone given as treatment for respiratory infections (TB)
        
        patients regulated for heinz bodies
      - NADP+
        
        Generated byGSH in red cells
        
        GSH reduced and oxidised by GSH peroxidase
        
        increase in h2o2 if defect in pathway
      - G6PD deficiency
        
        Sex linked on X chromosome
        
        heterozygous females protected against malaria falciparum
  - - - FBC shows less red cells
      - increases in reticulocytes & LDH
      - raised billirubin (conjugated and unconjugated)
      - decreased haptoglobin
      - Primiquine causes lots of h2o2 in acute cases
- - - - cant prevent complement so get intravascular haemolysis detected via flow cytometer
    - - eculizumab - inhibits complemenft via cs inhibition