Glucose-6-Phosphate Dehydrogenase Deficiency

Glucose-6-Phosphate Dehydrogenase Deficiency is a topic covered in the Washington Manual of Medical Therapeutics.

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General Principles

G6PD deficiency represents the most common disorder of RBC metabolism worldwide. Deficiency of G6PD renders RBCs more susceptible to oxidative damage through decreased glutathione reduction, leading to chronic or acute episodic hemolysis in the presence of oxidative stress.

Classification

More than 400 variants of G6PD are recognized. The severity of hemolysis depends on the degree of deficiency present.1

  • Milder forms, such as those seen in men of African heritage in the United States, result in self-limiting acute hemolytic episodes.
  • More severe forms, such as the Mediterranean variant, can result in severe hemolysis.
  • The most severe type causes a chronic, hereditary, nonspherocytic hemolytic anemia in the absence of an inciting cause.

Epidemiology

  • X-linked inheritance; thus degree of involvement in females is dependent on lyonization.
  • G6PD is felt to be protective against malaria, thus accounting for its prevalence in malaria-endemic areas.
  • Hemolysis is triggered by exposure to mediators of oxidative stress (i.e., drugs [see Table 21-5]), infections, and fava beans. Patients being considered for medications that trigger G6PD-dependent hemolysis should be tested for a deficiency before starting the drug.

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