Glucose-6-phosphate deficiency

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

Anemia

RBC Loss

• Hemorrhage

RBC consumption (Destruction/hemolytic)

• Hereditary
• Acquired
• Microangiopathic Hemolytic Anemia (MAHA)
• Autoimmune hemolytic anemia

Impaired Production (Hypochromic/microcytic)

• Iron deficiency
• Anemia of chronic disease
• Thalassemia
• Sideroblastic anemia

Aplastic/myelodysplastic (normocytic)

• Marrow failure
• Chemicals (e.g. ETOH)
• Radiation
• Infection (HIV, parvo)

Megaloblastic (macrocytic)

• Vitamin B12/folate deficiency
• Drugs (chemo)
• HIV

Management

• Identify and discontinue precipitating agent
• Supportive care for anemia, with transfusions rarely needed
• Hemolysis usually self-limited, resolving within 8-14 days
• Infants
  • Prolonged neonatal jaundice due to G6PD deficiency may require phototherapy
  • Exchange transfusion for severe neonatal jaundice

Precipitate and Avoid

• Infection
• Fava Beans
• Medications
  • Nitrofurantoin
  • Phenazopyridine
  • Dapsone
  • Chloramphenicol
  • Antimalarials
  • Sulfonamides
  • Ciprofloxacin, norfloxacin
  • Methylene blue
  • Vitamin K analogues
  • NSAIDs

Alternative Antibiotics

• Cephalexin (Keflex)

Also See

• Anemia
• Malaria

Source

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