title: [Successful management of orthopedic operations requiring general anesthesia in a PNH patient after introduction of eculizumab].
abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal hematopoietic stem cell disorder that presents with hemolytic anemia, thrombosis, and bone marrow failure. Stressors such as infection and pregnancy have been known to exacerbate hemolysis in PNH patients. Surgery can also trigger prominent complement activation and is an important risk factor for hemolysis. Furthermore, the postoperative thrombosis risk is high. Eculizumab, which is a humanized monoclonal antibody against C5, suppresses hemolysis and prevents thrombosis, and thus improves quality of life for PNH patients. However, few reports have focused on eculizumab-treated PNH patients undergoing surgery. We report a 79-year-old PNH patient receiving eculizumab treatment who underwent three consecutive orthopedic surgeries requiring general anesthesia. Perioperative management was carried out routinely, as in non-PNH patients, and no postoperative complications developed. Surgery was formerly considered to be a high risk event for PNH patients, but this case raises the possibility that even elderly PNH patients may undergo surgery safely when maintained on eculizumab treatment.
authors.affiliation: Division of Hematology, Department of Medicine, Juntendo University School of Medicine.
authors.forename: Miyuki
authors.initials: M
authors.lastname: Tsutsui
authors.forename: Akihiko
authors.initials: A
authors.lastname: Gotoh
authors.forename: Hajime
authors.initials: H
authors.lastname: Yasuda
authors.forename: Eriko
authors.initials: E
authors.lastname: Ono
authors.forename: Masaru
authors.initials: M
authors.lastname: Tanaka
authors.forename: Norio
authors.initials: N
authors.lastname: Komatsu
mesh_terms: D000368:Aged; D000768:Anesthesia, General; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000175:diagnosis / Q000188:drug therapy / Q000601:surgery*; D006801:Humans; D013927:Thrombosis / Q000188:drug therapy; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab