title: [A case of atypical hemolytic uremic syndrome successfully weaned from plasma exchange by treatment with eculizumab].
abstract: The patient was a 48-year-old man hospitalized for jaundice and anemia after a 6-day history of diarrhea. Examination demonstrated hemolytic anemia, renal dysfunction, and thrombocytopenia. Typical hemolytic uremic syndrome (HUS) was suspected based on the preceding colitis; however, plasma exchange (PE) was performed because the possibility of atypical HUS (aHUS) could not be ignored, given that the patient was an adult male. After 4 days of PE, his laboratory results improved. Stool culture on admission yielded negative results for Escherichia coli serotype O157 and ADAMTS13 activity. Antinuclear antibodies were normal, and no other drugs or infections indicating HUS were detected. Four months after onset, he suffered recurrence of aHUS after colitis. As a result, aHUS was suspected and therefore, PE was performed on the day of hospitalization. We diagnosed aHUS due to a result indicating complement dysregulation on hemolytic assay testing, which detected a complement factor H abnormality. After undergoing PE and maintaining a stable condition, the interval between PEs was extended; however, on day 17 after the last PE, he suffered a recurrent aHUS attack again. He could not be weaned from PE and started showing an allergic reaction to PE treatment, thereby leading to a switch from PE to eculizumab. Since switching to eculizumab treatment, the patient has not experienced another aHUS attack and his condition remains stable.
authors.forename: Akiko
authors.initials: A
authors.lastname: Nagata
authors.forename: Atsuko
authors.initials: A
authors.lastname: Ohara
authors.forename: Daisuke
authors.initials: D
authors.lastname: Wakasugi
authors.forename: Chikei
authors.initials: C
authors.lastname: Natori
authors.forename: Sakuya
authors.initials: S
authors.lastname: Ito
authors.forename: Kensei
authors.initials: K
authors.lastname: Taguchi
authors.forename: Kei
authors.initials: K
authors.lastname: Fukami
authors.forename: Seiya
authors.initials: S
authors.lastname: Okuda
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome; D017242:Complement Factor H / Q000652:urine; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D055502:Secondary Prevention; D016896:Treatment Outcome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D017242:Complement Factor H; C481642:eculizumab