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:
- broad/fava beans
During a crisis a blood film will show:
- bite cells
- heinz bodies (=inclusions within RBCs of denatured haemoglobin)
Management is to avoid precipitants and transfuse if necessary.
On to Kaposi's sarcoma...