유전성 구상 적혈구증은 적혈구 골격 형성 장애로 인하여 황달과 빈혈을 유발할 수 있는 질환이다. 국내 영유아 검진 및 국가예방접종사업 등으로 인하여 대부분 성인이 되기 전에 병의 진단 및 치료가 잘 이루어지고 있다. 하지만 빈혈, 비장종대를 동반한 성인 환자에서 간경변증의 증거 없이 간수치 상승이 동반되는 경우는 드물지만 유전성 구상 적혈구증 또한 감별진단에 포함시켜야 하며, 담관결석에 대한 가능성을 염두에 두어야 한다.
Hereditary spherocytosis is a disease caused by deficiency of erythrocyte lipid membrane protein. Hereditary spherocytosis shows hemolysis of erythrocyte, and it leads to anemia, jaundice by elevation of indirect bilirubin. Almost of patients are diagnosed in their infancy, and can be cured by splenectomy about their age 6–7. Herein, we report a rare case of 33-year-old male was suffered from gallbladder stone and cholangitis those are thought to be the late complications of hereditary spherocytosis. We performed endoscopic retrograde cholangiopancreaticography to remove common bile duct stones. After he got cholecystectomy and splenectomy, there was no recurrence of choledocholithiasis. This is the first case in Korea who didn’t undergo splenectomy until grown up, shows cholangitis as a late disease manifestation of hereditary spherocytosis.