Patient's question:
When does favism occur with hemolysis? Can it happen even without taking any medication? If not, will a routine urinalysis test show occult blood? Can hemolysis be detected if it's not severe?Doctor's answer:
Sickle cell disease is a disease caused by a deficiency of glucose-6-phosphate dehydrogenase (G-6-PD), characterized by sudden intravascular hemolysis after consuming fresh fava beans in the context of hereditary glucose-6-phosphate dehydrogenase (G-6-PD) deficiency. Concurrent with hemolytic anemia, symptoms such as vomiting, diarrhea, and abdominal pain worsen, accompanied by hepatomegaly and abnormal liver function. Approximately 50% of patients develop splenomegaly. Severe cases may present with coma, convulsions, and acute kidney failure, and if emergency treatment is not administered promptly, death often occurs within 1 to 2 days.