Favism

Overview

Favism is an inherited hemolytic disorder caused by glucose-6-phosphate dehydrogenase (G6PD) deficiency, in which patients develop hemolysis after eating fava beans or being exposed to certain oxidative substances. It presents as acute intravascular hemolysis with jaundice, anemia, and dark-colored urine.

Symptoms

• Acute hemolysis after eating fava beans
• Jaundice
• Soy sauce-colored urine
• Pallor
• Fatigue

Treatment

Treatment centers on avoiding triggers and symptomatic support during the acute hemolytic phase; severe hemolysis requires transfusion, and patients should lifelong avoid fava beans and related medications.

Dietary advice

Strictly avoid eating fava beans and their products, use hemolysis-inducing drugs and foods with caution, and otherwise maintain a balanced, nutritious diet.