title: Perisurgical induction of eculizumab in a patient with paroxysmal nocturnal hemoglobinuria: its inhibition of surgery-triggered hemolysis and the consequence of subsequent discontinuation.
abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by complement (C')-induced lysis of PNH red blood cells (RBCs), which are deficient in the expression of CD55 and CD59. Surgery is one of the major clinical situations that trigger hemolytic attack and thrombosis in PNH. We describe here a case of 64-year-old man with classic PNH complicated by early-stage gastric cancer requiring distal gastrectomy under general anesthesia. We administered humanized monoclonal anti-C5 antibody (eculizumab; Soliris) for a limited period (600 mg, once a week × four times) perisurgically. Eculizumab effectively inhibited the C' system and the patient underwent a curative distal gastrectomy without significant surgery-triggered hemolytic attack. Although discontinuation of eculizumab induced mild hemolysis 2 weeks after the last administration, it was treated conservatively without thrombotic complication. Limited-term induction of eculizumab could be an option for PNH patients with transient and anticipated high risks, with careful preparation for the discontinuation-related risks afterwards.
authors.affiliation: Department of Hematology bDepartment of Surgery, University of Tsukuba, Tsukuba, Japan. kuripon@mvb.biglobe.ne.jp
authors.forename: Naoki
authors.initials: N
authors.lastname: Kurita
authors.forename: Naoshi
authors.initials: N
authors.lastname: Obara
authors.forename: Kuniyoshi
authors.initials: K
authors.lastname: Fukuda
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Nishikii
authors.forename: Shoko
authors.initials: S
authors.lastname: Sato
authors.forename: Satoshi
authors.initials: S
authors.lastname: Inagawa
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Kurokawa
authors.forename: Yohei
authors.initials: Y
authors.lastname: Owada
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Ninomiya
authors.forename: Shigeru
authors.initials: S
authors.lastname: Chiba
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage* / Q000009:adverse effects; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood* / Q000188:drug therapy / Q000601:surgery*; D006461:Hemolysis / Q000187:drug effects; D006801:Humans; D008297:Male; D008875:Middle Aged; D011300:Preoperative Care / Q000379:methods
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab