Sickle cell anemia is a severe hemolytic disease caused by a congenital hemoglobin defect for which there is no treatment. Clinical management of patients with the disorder is purely symptomatic, and a "hands off" policy regarding transfusion is best. It is unusual to be able to offer any definitive treatment which holds promise for permanent improvement. Although rarely indicated, splenectomy can afford lasting benefit in the properly selected individual. Two such patients are reported.
—This Negro man was seen many times between the ages of 5 and 12 years at King County Hospital, Seattle, for complications of sickle cell anemia. Hemoglobin levels were between 4.0 and 7.6 gm/100 ml. Reticulocytes ranged up to 14%. Sickling preparations gave positive reactions and hemoglobin electrophoresis showed a single S band. The spleen was reported as descending to the umbilicus when he was 5 years old and was below this
Stevens AR. Splenectomy in Sickle Cell Anemia. Arch Intern Med. 1970;125(5):883-884. doi:10.1001/archinte.1970.00310050121016