What is the management and treatment of Favismo (Glucose-6-phosphate dehydrogenase deficiency-induced hemolytic anemia)?

Management and Treatment of Favism (G6PD Deficiency-Induced Hemolytic Anemia)

Immediate Management

The cornerstone of favism management is immediate cessation of fava bean exposure, aggressive hydration therapy, and blood transfusion when hemoglobin drops to dangerous levels, with most patients requiring hospitalization for monitoring. 1, 2

Acute Hemolytic Crisis Management

Hydration/Fluid Therapy:

• Intravenous hydration is the primary treatment modality, used in approximately 68% of pediatric cases 1
• Aggressive fluid therapy prevents acute kidney injury from free hemoglobin deposition and maintains adequate renal perfusion 2
• Continue hydration until hemoglobinuria resolves and hemoglobin stabilizes 2

Blood Transfusion:

• Required in approximately 36% of favism cases when anemia becomes severe 1
• Transfuse packed red blood cells at approximately 18.9 cc/kg (though accurate calculation is critical to avoid over-transfusion) 1
• Transfusion is indicated for symptomatic anemia, cardiovascular compromise, or hemoglobin levels threatening organ perfusion 2
• Most cases require hospitalization and transfusion despite self-limited symptomatology 2

Adjunctive Therapy:

• Human haptoglobin administration can be effective for relieving bilirubinaemia and hemoglobinuria during acute crisis 3
• Haptoglobin rapidly decreases serum-free hemoglobin to normal levels even when lactate dehydrogenase remains elevated 3
• This represents a novel therapeutic option for managing acute hemolytic crisis 3

Clinical Monitoring

Laboratory Surveillance:

• Monitor complete blood count with hemoglobin determination at baseline and serially during crisis 4, 5
• Track bilirubin levels (both direct and indirect), lactate dehydrogenase, haptoglobin, and reticulocyte count 6
• Assess for hemoglobinuria and urinary urobilinogen 2
• Monitor renal function to detect acute kidney injury from hemoglobinuria 2

Warning Signs Requiring Immediate Intervention:

• Dark reddish-brown urine (hemoglobinuria) 5, 2
• Marked decrease in hemoglobin 5
• Jaundice (present in 82.5% of cases) 1, 2
• Pallor, tachycardia, abdominal pain, malaise 2
• Back or abdominal pain suggesting acute hemolysis 4

Variant-Specific Considerations

Mediterranean Variant (G6PD Med):

• Causes the most severe hemolytic episodes with life-threatening potential 4, 7
• Associated with significantly greater anemia severity compared to other variants 8
• Over-represented in favism cases, indicating higher susceptibility 8
• Requires more aggressive monitoring and earlier intervention 8

Cairo Variant (G6PD Cairo):

• Produces severe anemia comparable to Mediterranean variant 8
• Characterized by greater hyperbilirubinemia than other variants 8
• Unique feature: persistence of mild anemia and reticulocytosis for up to 4 months post-recovery 8
• Requires extended follow-up beyond acute crisis resolution 8

African Variant (G6PD A-):

• Associated with milder, self-limited hemolysis 4, 5
• Under-represented in severe favism cases 8
• Less severe anemia compared to Mediterranean and Cairo variants 8
• Still requires standard acute management but generally better prognosis 8

Age-Specific Manifestations

Infants and Pregnant Women:

• Highest severity with risk of death in exclusively breastfed infants exposed through maternal milk 2
• Systolic murmur may develop 2
• Requires most aggressive monitoring and intervention 2

Children (weaned to 11 years):

• Visual impairment can occur uniquely in this age group 2
• Systolic murmur possible 2
• Most common age group for favism presentation 1

Adults:

• Renal failure is a specific complication in this age group 2
• Generally less severe than pediatric cases but still requires hospitalization 2

Prevention and Long-Term Management

Mandatory G6PD Testing:

• Screen all individuals with predisposing racial or ethnic backgrounds upon healthcare entry 4
• Test before initiating any oxidant drug therapy 4, 7
• Qualitative screening for initial assessment, quantitative testing when specific medications (like tafenoquine) are considered 4, 7

Strict Avoidance:

• Complete avoidance of fava beans in all forms (fresh, dried, cooked) 2, 8
• Avoid oxidant medications including primaquine, dapsone, methylene blue, and sulfonamides 4, 7
• Exercise caution with local anesthetics (benzocaine, prilocaine, lidocaine, tetracaine) 7

Family Screening:

• Test all first-degree relatives of diagnosed patients 4, 5
• Provide genetic counseling regarding X-linked inheritance pattern 5

Common Pitfalls

• Incorrect blood transfusion calculations increase transfusion frequency and prolong hospitalization 1
• Delayed recognition of hemolysis when fava bean exposure history is not initially obtained 9
• Premature discharge before confirming hemolysis resolution, particularly with Cairo variant requiring 4-month monitoring 8
• Using methylene blue for methemoglobinemia in G6PD-deficient patients worsens hemolysis; use ascorbic acid (0.2-1.0 g/day orally) instead 7