Definitive treatment of Hereditary Spherocytosis?

• A. Cholecystectomy
• B. Bone Marrow Transplant
• C. Splenectomy
• D. IVIG

Answer: (C)

Hereditary spherocytosis is caused by defects in red cell membrane proteins that make red cells spherical and prone to being destroyed by the spleen. The spleen is the main site where these rigid spherocytes are sequestered and removed, so removing the spleen markedly decreases ongoing hemolysis and often corrects the anemia and jaundice. That is why splenectomy is considered the definitive treatment. Cholecystectomy may be needed if pigment gallstones develop from chronic hemolysis, but it does not address the underlying hemolysis. Bone marrow transplant isn’t used for this condition, and IVIG is not indicated for hereditary spherocytosis. After splenectomy, there’s an increased risk of infection from encapsulated organisms, so appropriate vaccination and precautions are important.