G6PD Deficiency

 

 

Background 

 

  • X-linked disorder, the most common enzymatic disorder of RBCs

  • Hemolysis is the result of the inability of the RBC to protect itself from oxidative stress

  • Spectrum of disease: chronic hemolysis, intermittent hemolysis, hemolysis only with triggers, no hemolysis 

  • Acute insults that either precipitate or aggravate hemolysis include infection, certain drugs, & fava beans 

 

 

Considerations 

 

  • Chronic hemolysis:

    • Chronic pRBC transfusions 

    • Difficult crossmatch 

    • Anemia

  • Avoidance of oxidative stress as it precipitates acute hemolysis:

    • Oxidative drugs (see list below)

    • Infection, hypoxia, hypothermia, stress

    • Metabolic abnormalities (e.g., diabetic ketoacidosis)

    • Foods (e.g., fava beans)

  • Unable to reduce methemoglobin: 

    • Methemoglobinemia: oxidative stress

    • Methylene blue: oxidative stress

  • Consider hematology consultation 

 

 

Goals 

 

  • Identify patients at risk

  • Avoid precipitants of oxidative stress

  • Avoid precipitants of methemoglobinemia

  • Manage hemolysis by removing trigger, pRBC transfusion as needed, supportive management

 

 

Common Drugs to Avoid 

 

  • Acetaminophen

  • Acetylsalicylic acid

  • Ciprofloxacin

  • Dapsone

  • Methylene blue

  • Nitrofurantoin

  • Phenytoin

  • Streptomycin

  • Sulpha drugs

 

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