BOF: General (Red Cell)

Which ONE of the following statements regarding glucose-6-phosphate dehydrogenase (G6PD) deficiency is true?

a) Dapsone can be safely given in G6PD deficiency

b) G6PD is inherited in an autosomal dominant fashion

c) G6PD levels are best measured during the acute phase of a haemolytic crisis

d) Heinz bodies are seen on the MGG peripheral blood film

e) In favism, the degree of haemolysis is dose related to the amount of fava bean ingested.

The Answer:

e) In favism, the degree of haemolysis is dose related to the amount of fava bean ingested.

Explanation:

G6PD is inherited in an X-linked manner, although sporadic cases are reported. Heinz bodies can be seen on a Brilliant cresyl blue stain. If G6PD assays are done in the acute haemolytic phase, false negative results may be obtained if the reticulocytes are increased. Favism occurs following ingestion of fava beans and cause a severe haemolysis and renal failure. The severity is dose related to the amount ingested. There is a long list of drugs which are known to precipitate an oxidative crisis, one of which is dapsone.

Reference:

Beutler E. Glucose-6-phosphate dehydrogenase deficiency: a historical perspective. Blood. 2008 Jan 01;111:16–24