Hereditary spherocytosis (HS) is a familial hemolytic disorder associated with a variety of mutations that lead to defects in red blood cell (RBC) membrane proteins. Splenectomy is the definitive treatment for HS. Patients with very mild HS may remain unaffected by their disorder unless challenged by an environmental stressor. In patients who undergo splenectomy, RBC survival improves dramatically, and most are able to maintain a normal hemoglobin level.