Cholelithiasis with a cholecystoduodenal fistula complicated with paroxysmal nocturnal hemoglobinuria

In cases of paroxysmal nocturnal hemoglobinuria ( PNH ), attention must be paid to potential complications such as thrombosis and hemolysis due to perioperative stress and infection from complement activation . Here we present the case of a 61- year - old Japanese woman with PNH . We made the diagnosis of PNH when she was 28 years old , and we administered repeated steroid medication and erythrocyte transfusion . The patientʼs cholecystocholedocholithiasis with a cholecystoduodenal fistula was diagnosed based on a survey of the right hypochondriac pain . We performed endoscopic nasobiliary drainage ( ENBD ) for the prophylaxis of perioperative infection , plus a cholecystectomy and fistulectomy . There were no complications , including hemolysis attack , infection , thrombosis with irrigation erythrocyte transfusion , steroid cover , or the need for heparin administration during the perioperative period . The reduction of the complement activation is necessary in the perioperative management of PNH patients . The prevention of the development of acidosis and hypoxemia , the selection of washed red blood cells , steroid use , appropriate infection measures and thrombosis prophylaxis are all important for the prevention of complications .