Saturday, 9 October 2010

MRCP revision battle 28.4: G6PD deficiency

Glucose-6-phosphate dehydrogenase deficiency is the commonest RBC enzyme defect.

It is x-linked recessive.

It causes intravascular haemolysis.

Generally patients are asymptomatic unless a crisis is precipitated, in which case they become anaemic and jaundiced.

Precipitants of crisis include:
  • drugs
    • primaquine
    • sulfonamides
    • ciprofloxacin
    • aspirin
  • broad/fava beans
  • illness

During a crisis a blood film will show:
  • bite cells
  • heinz bodies (=inclusions within RBCs of denatured haemoglobin)
 click here for a whole page of images of Heinz bodies

 Management is to avoid precipitants and transfuse if necessary.

On to Kaposi's sarcoma...