🧪 G6PD Deficiency: The Hemolytic Anemia Triggered by Stress
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzymatic disorder of red blood cells—affecting over 400 million people worldwide.
It causes hemolytic anemia in response to oxidative stress, and is a common vignette trigger in both Step 1 (enzymes, metabolism) and Step 2 (clinical diagnosis and management).
USMLE Tip: Think G6PD any time hemolysis follows infection, sulfa drugs, or fava beans.
🧬 Pathophysiology
G6PD is key in the pentose phosphate pathway, producing NADPH
NADPH maintains glutathione, which protects RBCs from oxidative damage
Without G6PD → ↑ oxidative stress → RBC membrane damage → intravascular hemolysis
🔥 Common Triggers
Trigger Type | Examples |
---|---|
Infections | Pneumonia, hepatitis, malaria |
Drugs | Sulfonamides, dapsone, antimalarials, nitrofurantoin |
Foods | Fava beans |
Stress | DKA, hypoxia, surgery |
🧪 Lab Findings
Test | Result |
---|---|
Hemoglobin | ↓ |
LDH | ↑ |
Haptoglobin | ↓ |
Reticulocyte count | ↑ |
Peripheral Smear | Bite cells, Heinz bodies |
G6PD assay | ↓ (not during acute crisis) |
🩸 Peripheral Smear Findings
Heinz bodies → denatured hemoglobin (seen with supravital stain)
Bite cells → splenic macrophage removal of Heinz bodies
👀 Buzzwords for USMLE: "Back from vacation in Greece," "ate fava beans," "sudden jaundice after sulfa antibiotics."
🧑⚕️ Management
Avoid triggers (especially offending drugs)
Supportive care: hydration, oxygen
Rarely transfusion if severe
Genetic counseling in affected families
📚 Sample USMLE Vignette
A 24-year-old male of Mediterranean descent develops jaundice and dark urine after taking trimethoprim-sulfamethoxazole for a UTI. Blood smear shows bite cells. What’s the most likely diagnosis?
✅ Answer: G6PD deficiency
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We’ll now generate your 1200x800 feature image with a visual breakdown of the enzyme pathway, triggers, smear findings, and treatment pearls.
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📲 Call to Action
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