Cold Agglutinin Disease Leading to Rapid Death after Open Liver Resection—A Case Report—

Abstract

Cold agglutinin disease (CAD) is the reversible agglutination of red blood cells at low temperatures, followed by hemolysis during rewarming and further aggregation that leads to thrombotic complications. An 83-year-old woman underwent anterior sectionectomy and partial resection of liver segment 2 for hepatocellular carcinomas (HCCs) during follow-up for chronic hepatitis C after having achieved sustained virological response (SVR). Although cold autoantibodies were detected on preoperative blood typing, there was no diagnosis of CAD at that time. On postoperative day (POD) 3, marked decreases in red blood cells and hematocrit occurred, and severe red blood cell agglutination appeared and persisted. On POD5, computed tomography (CT) performed for suspected bile leakage showed portal vein thrombosis and right hepatic lobe necrosis. Severe cyanosis developed on POD6, CAD was diagnosed, and treatment for CAD was initiated. However, the skin findings worsened, and the patient suddenly deteriorated the next day and died. Pathological autopsy showed severe agglutination of blood and separation of plasma. There have been no reports of such a sudden post-operative onset and extremely severe course as seen in this case. Therefore, this case is reported to sound the alarm.