Question
Under what circumstances does sickle cell anemia present with hemolytic symptoms? Can it occur without taking any medication? If not, would a routine urine test show occult blood? Can hemolytic symptoms be detected if they are not severe?
Answer
Sickle cell anemia is a disease caused by a deficiency in the enzyme 6-phosphogluconate dehydrogenase (g-6-pd), primarily manifested by acute intravascular hemolysis after consuming fresh beans. In addition to hemolytic anemia, symptoms may include vomiting, diarrhea, and worsening abdominal pain, liver enlargement, and abnormal liver function. Approximately 50% of patients may also have spleen enlargement. Severe cases may present with coma, seizures, and acute kidney failure, and death usually occurs within 1 to 2 days if emergency measures are not taken promptly.