Korean J Pancreas Biliary Tract.  2020 Jul;25(2):123-127. 10.15279/kpba.2020.25.2.123.

Cholangitis Caused by Hereditary Spherocytosis in Adulthood Treated by Endoscopic Retrograde Cholangiopancreatography

• Park TY
• Jun JH
• Kang H
• Doh YS
• Jang JW
• Baek IH
• Jung SH

• KMID: 2505003
• DOI: http://doi.org/10.15279/kpba.2020.25.2.123

Abstract

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.

Keyword

유전성 구상적혈구증; 담관염; 총담관담석; Spherocytosis; Hereditary; Cholangitis; Common bile duct calculi