A Safety Management Case of Laparoscopic Colectomy in a Patient With Paroxysmal Nocturnal Hemoglobinuria

Abstract

Paroxysmal nocturnal hemoglobinuria is a rare and life-threatening disorder of acquired hemolytic anemia. Surgery is one of the major clinical situations that trigger hemolytic attack. Eculizumab is a humanized monoclonal antibody that binds the complement protein C5 and prevents complement-mediated hemolysis via inhibition of the terminal complement cascade. A 76-year-old woman received a diagnosis of ascending colon cancer during the search for the cause of right lower abdominal pain. She had received a diagnosis of paroxysmal nocturnal hemoglobinuria and been followed for 26 years at our hospital. We planned to start eculizumab for perioperative management in order to reduce the risk of the patient developing hemolytic crisis as a result of surgery. We administered 600 mg of eculizumab on the 15th, 8th, and 1st preoperative days. The levels of serum complement and lactate dehydrogenase decreased with the first administration of eculizumab. Laparoscopic right hemicolectomy was performed successfully. The patient had good postoperative progress. We administered 600 mg of eculizumab on the 6th postoperative day and 900 mg of eculizumab on the 13th postoperative day. She was discharged from hospital on the 16th postoperative day. We started use of eculizumab before surgery for safety in the management of the operation and during the perioperative period. When we enforce the elective operation for patients with paroxysmal nocturnal hemoglobinuria who do not start treatment of eculizumab, we recommend the use of eculizumab for perioperative management of patients with paroxysmal nocturnal hemoglobinuria.