title: Atypical hemolytic uremic syndrome in first trimester pregnancy successfully treated with eculizumab.
abstract: BACKGROUND
  Atypical hemolytic uremic syndrome is a rare disorder which is known to cause acute thrombotic microangiopathy during pregnancy with poor maternal and fetal outcomes. Atypical hemolytic uremic syndrome is caused mostly by dysregulation of alternative complement pathway secondary to genetic mutations. Most of the cases reported have been in the post-partum period. We report a rare case of a patient who presents with thrombotic microangiopathy in the first trimester of her eleventh pregnancy and was successfully treated with eculizumab.
  
  
  METHODS
  A 30-year-old woman presented at 10 weeks of gestation with hypertension, hemolytic anemia, thrombocytopenia, and acute kidney injury, consistent with thrombotic microangiopathy. She was managed initially with daily plasmapheresis. However, her kidney function did not recover, requiring hemodialysis. ADAMTS13 activity was later found to be within normal limit, hence diagnosis of atypical hemolytic uremic syndrome was strongly considered at that time and she was immediately treated with anti-C5 humanized monoclonal antibody (eculizumab). The patient responded well (resolution of thrombotic microangiopathy and recovery of renal function) to eculizumab, with continued remission after discharge and successfully delivered a healthy baby at term without any peripartum complications.
  
  
  CONCLUSIONS
  Early recognition of atypical hemolytic uremic syndrome is often difficult as several other conditions also manifest as thrombotic microangiopathy during pregnancy, causing delay in initiating appropriate treatment. Our case suggests that treatment of atypical hemolytic uremic syndrome in early trimester of pregnancy with eculizumab results in good outcome to mother and fetus.
authors.affiliation: Division of Internal Medicine, New York Medical College, Westchester Medical Center, Valhalla, NY 10595 USA.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Andries
authors.affiliation: Division of Internal Medicine, New York Medical College, Westchester Medical Center, Valhalla, NY 10595 USA.
authors.forename: Michael
authors.identifier: 0000-0002-3222-5664
authors.initials: M
authors.lastname: Karass
authors.affiliation: Division of Internal Medicine, New York Medical College, Westchester Medical Center, Valhalla, NY 10595 USA.
authors.forename: Srikanth
authors.initials: S
authors.lastname: Yandrapalli
authors.affiliation: Division of Internal Medicine, New York Medical College, Westchester Medical Center, Valhalla, NY 10595 USA.
authors.forename: Katherine
authors.initials: K
authors.lastname: Linder
authors.affiliation: Division of Hematology and Oncology, New York Medical College, Westchester Medical Center, Valhalla, NY 10595 USA.
authors.forename: Delong
authors.initials: D
authors.lastname: Liu
authors.affiliation: Division of Hematology and Oncology, New York Medical College, Westchester Medical Center, Valhalla, NY 10595 USA.
authors.forename: John
authors.initials: J
authors.lastname: Nelson
authors.affiliation: Division of Nephrology, New York Medical College, Westchester Medical Center, Valhalla, NY 10595 USA.
authors.forename: Rahul
authors.initials: R
authors.lastname: Pawar
authors.affiliation: Division of Nephrology, New York Medical College, Westchester Medical Center, Valhalla, NY 10595 USA.
authors.forename: Savneek
authors.initials: S
authors.lastname: Chugh
keywords: Atypical hemolytic uremic syndrome (aHUS); Eculizumab; First trimester; Pregnancy; Thrombotic microangiopathy (TMA)