A patient with paroxysmal nocturnal hemoglobinuria being treated with eculizumab who underwent laparoscopic cholecystectomy: report of a case

Paroxysmal nocturnal hemoglobinuria (PNH) is acquired hemolytic anemia characterized by symptoms such as anemia and hemoglobinuria. In recent years, eculizumab as an anti-complement (C5) monoclonal antibody has been used for PNH and shown to have marked effects. We performed laparoscopic cholecystectomy in a patient with PNH being treated with eculizumab, and could avoid the risk of perioperative hemolysis and thrombosis. [Patient] The patient was a 48-year-old female who had developed PNH when she was 39 years old. At the age of 46 years, eculizumab administration was initiated once every 2 weeks. During the administration period, neither the progression of anemia nor hemoglobinuria was observed. In March 2013, gallstones were detected, and she was referred to our hospital for surgery. Eculizumab was administered 10 days before surgery, and laparoscopic cholecystectomy was performed in May 2013. After the operation, for the prevention of thrombosis, elastic stockings and a foot pump were used without anticoagulant administration. After the operation, neither the progression of anemia nor hemoglobinuria was observed. On postoperative day 5, eculizumab was administered as planned, and she showed a favorable general condition and was discharged. [Discussion] Perioperative care in PNH patients was conventionally considered to involve a high risk of developing anemia, thrombosis, or infection. However, after the advent of eculizumab, the control of the symptoms of PNH became possible in many patients. In this patient with PNH being treated with eculizumab, safe perioperative management was possible without the development of complications.