Glucose-6-phosphate dehydrogenase deficiency

Glucose-6-phosphate dehydrogenase (G6PD) is an enzyme that reduces oxidative stress by reducing glutathione levels within red cells. G6PD deficiency is an X-linked disorder common in West Africa, southern Europe, the Middle East and south-east Asia. Patients are usually asymptomatic until oxidative stress causes haemolytic anaemia. Common triggers include fava broad beans (broad-bean induced haemolysis is termed favism), drugs (including antimalarials and sulphonamide antibiotics) and infections. Treatment is supportive, although transfusions may be required for acute haemolytic crises. Heinz bodies are denatured haemoglobin aggregates that are found in the cytoplasm of red blood cells. They result from oxidant stress (e.g. G6PD deficiency) or haemolysis. Recessive carriers of the G6PD mutation are protected against malaria.