Glucose-6-phosphate deficiency

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Background

  • X-Linked recessive; protects against Malaria
  • African, Asian, and Mediterranean descent
  • Nonimmune mediated hemolytic anemia
  • Stress or drugs can cause hemoglobin precipitation within the RBC
    • Leads to removal of the cell from circulation via the spleen

Clinical

  • Fatigue
  • Hemolytic anemia
  • Jaundice
  • Splenomegaly

Complications

  • Severe hemolysis and anemia
  • Cardiovascular collapse

Labs

  • CBC
    • Heinz Bodies
  • Retic Count
    • Retic count high
      • Coombs negative: G6PD, SCD, spherocytosis, microangiopathic hemolysis

DDx

Precipitate and Avoid

  • Infection
  • Medications
    • Nitrofurantoin
    • Phenazopyridine
    • Dapsone
    • Chloramphenicol
    • Antimalarials
    • Sulfonamides
  • Fava Beans

Alternative Antibiotics

  • Cephalexin (Keflex)

Also See

Source

  • Tintinnalli, 7th edition, Chapter 231, “Sickle Cell and Other Hereditary Hemolytic Anemias,” accessed via Access Emergency Medicine
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