Anesthetic management of a patient with paroxysmal nocturnal hemoglobinuria

A 34-year-old man with paroxysmal nocturnal hemoglobinuria (PNH) was scheduled for emergency laparotomy. PNH is an acquired disorder of stem cells, and the common manifestations are complement mediated hemolytic anemia and deep venous thrombosis. Perioperative hemolysis occurs under the activation of complement induced by stress such as acidosis, infection, and insufficient pain control. Activation of complement secondary leads to platelet aggregation and hypercoagulability. We administrated remifentanil for the pain control during the operation and fentanyl after the operation. We avoided hypoventilation and dehydration to prevent acidosis. Washed red blood cells were given to reduce the chance of complement activation and we administrated low molecular weight heparin up to the seventh postoperative day to prevent deep venous thrombosis. The perioperative course was uneventful without complication.