hematology and oncology
Glucose 6 Phosphate Dehydrogenase (G6PD) Deficiency
Pathophysiology of G6PD Deficiency
G6PD deficiency renders RBCs susceptible to oxidative stress, which shortens RBC survival. Hemolysis occurs following an oxidative challenge, commonly after fever, acute viral or bacterial infections, and Diabetic Ketoacidosis (DKA) . Hemolysis is episodic and self-limited, although rare patients have chronic, ongoing hemolysis in the absence of oxidative challenge. Less commonly, hemolysis occurs after exposure to drugs or to other substances that produce peroxide and cause oxidation of hemoglobin and RBC membranes. These drugs and substances includeprimaquine, salicylates, sulfonamides, nitrofurans, phenacetin, naphthalene, some vitamin K derivatives,dapsone,phenazopyridine, nalidixic acid,methylene blue, and, in some cases, fava beans. The amount of hemolysis depends on the degree of G6PD deficiency and the oxidant potential of the drug. 