title: Innovative therapeutic approach: sequential treatment with plasma exchange and eculizumab in a pregnant woman affected by atypical hemolytic-uremic syndrome.
abstract: The atypical HUS (aHUS) is a rare genetic disease, with poor prognosis, characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. This syndrome is often related to mutations in the genes encoding complement regulatory proteins. A 26-year-old woman with homozygous mutation in complement factor H (CFH) developed a relapse of aHUS at 17th week of pregnancy. Despite treatment with plasma exchange (PEX), at the 26th week of gestation eculizumab was started. The sequential treatment with eculizumab after PEX was well tolerated and it has led to clinical remission.
authors.affiliation: U.O. Immunohematology and Transfusion Medicine, Rimini AUSL Romagna. Electronic address: mmussoni@auslrn.net.
authors.forename: Maria Pia
authors.initials: MP
authors.lastname: Mussoni
authors.affiliation: U.O. Immunohematology and Transfusion Medicine, Rimini AUSL Romagna.
authors.forename: F A
authors.initials: FA
authors.lastname: Veneziano
authors.affiliation: U.O. Immunohematology and Transfusion Medicine, Rimini AUSL Romagna.
authors.forename: L
authors.initials: L
authors.lastname: Boetti
authors.affiliation: U.O. Immunohematology and Transfusion Medicine, Rimini AUSL Romagna.
authors.forename: C
authors.initials: C
authors.lastname: Tassi
authors.affiliation: U.O. Immunohematology and Transfusion Medicine, Rimini AUSL Romagna.
authors.forename: C
authors.initials: C
authors.lastname: Calisesi
authors.affiliation: U.O. Immunohematology and Transfusion Medicine, Rimini AUSL Romagna.
authors.forename: S
authors.initials: S
authors.lastname: Nucci
authors.affiliation: U.O. Nephrology and Dialysis, Rimini AUSL Romagna.
authors.forename: A
authors.initials: A
authors.lastname: Rigotti
authors.affiliation: U.O. Quality, Research and Innovation, Rimini AUSL Romagna.
authors.forename: I
authors.initials: I
authors.lastname: Panzini
authors.affiliation: Centre for the Care and Study of HUS, Ospedale Maggiore Policlinico Milan.
authors.forename: G
authors.initials: G
authors.lastname: Ardissino
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D065766:Atypical Hemolytic Uremic Syndrome / Q000235:genetics / Q000628:therapy*; D017242:Complement Factor H / Q000235:genetics; D005260:Female; D030342:Genetic Diseases, Inborn / Q000235:genetics / Q000628:therapy*; D006720:Homozygote; D006801:Humans; D009154:Mutation; D010951:Plasma Exchange; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000235:genetics / Q000628:therapy*
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D017242:Complement Factor H; C481642:eculizumab
keywords: Atypical HUS; Eculizumab; Transfusion practices