• Young woman with a family history of anemia presents with jaundice, splenomegaly and anemia with normal reticulocyte count, negative Coombs test and elevated mean corpuscular hemoglobin concentration with a peripheral smear showing spherocytes (RBCs without area of central pallor), most consistent with hereditary spherocytosis
• Key idea: Hereditary spherocytosis is primarily transmitted in an autosomal dominant fashion and is treated with splenectomy (because the hemolysis occurs in the spleen because the splenic macrophages recognize the spherocytes as being misshapen)

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Hereditary Spherocytosis – spleen removes spherocytes (anemia). Fragile RBCs are hemolyzed (jaundice).

• *Elevated MCHC = Hereditary Spherocytosis
• Peripheral smear – round RBCs, no central pallor
• Splenectomy is curative