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G6PD deficiency

  1. Clinical features                                     
    Acute drug- induced hemolysis
Drugs that Carry Risk of Clinical Hemolysis in Persons with G6PD Deficiency
  Definite Risk Possible Risk Doubtful Risk
Antimalarials Primaquine Chloroquine Quinine
Sulphonamide Sulphametoxazole Sulfasalazine Sulfisoxazole
  Others Sulfadimidine Sulfadiazine
Antibacterial/antibiotics Cotrimoxazole Ciprofloxacin Chloramphenicol
  Nalidixic acid Norfloxacin p-Aminosalicylic acid
Antipyretic/analgesics Acetanilide Acetylsalicylic acid high dose (>3 g/d) Acetylsalicylic acid <3 g/d
  Phenazopyridine (Pyridium)   Acetaminophen
Other Naphthalene Vitamin K analogues Doxorubicin
  Methylene blue Ascorbic acid >1 g Probenecid
  1. Laboratory features
    Non-spherocytic intravascular hemolysis during an attack
   The blood film will show:
  1. Bite cells (Red cells with a ‘bite’ of membrane missing)
  2. Blister cells (Red cells with surface blistering of the membrane)
  3. Irregularly shaped small cells
  4. Polychromasia reflecting the reticulocytosis
  5. Denatured haemoglobin visible as Heinz bodies within the red cell cytoplasm, if stained with a supravital stain such as methyl violet
G6PD level
  1. Can be indirectly assessed by screening methods which usually depends upon the decreased ability to reduce dyes.
  2. Direct assessment of G6PD is made in those with low screening values
  3. Care must be taken close to an acute hemolytic episode because reticulocytes may have normal enzyme levels and give rise to a false normal result.

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