2025 – PAGE 282 – HEMATOLOGY & ONCOLOGY

(DOUBLE TAKE) GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY (G6PD DEFICIENCY)

Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD Deficiency) is an X-linked recessive disorder (so look for a male patient!) resulting in jaundice, dark urine, and anemia due to hemolysis from oxidative injury. In newborns, this can present with jaundice in a MALE baby within the first 24 HOURS of life. In other children, it can result in hemolysis after ingestion of fava beans, malaria medications, trimethoprim-sulfamethoxazole, ciprofloxacin, or nitrofurantoin. Look for HEINZ BODIES (purple granules noted in the red cells on microscopy). G6PD Deficiency is associated with patients of African American and Mediterranean descent.

PEARL: Do not test for the deficiency during the acute hemolytic phase. Instead, wait a few weeks because a false negative result can occur due to the build-up of G6PD in reticulocytes.

MNEMONIC: Imagine a bottle of HEINZ BODIES ketchup that has an X located on the exact spot that you have to hit it to make the broken RBCs come out of the bottle. Or, look at this X-shaped HEINZ BODIES bottle of ketchup.

NAME ALERT: This is not the same as glucose-6-phosphatase deficiency, which can affect glucose metabolism and is associated with glycogen storage disease 1, von Gierke’s disease.