title: Postpartum thrombotic microangiopathy revealed as atypical hemolytic uremic syndrome successfully treated with eculizumab: a case report.
abstract: BACKGROUND
  Differential diagnosis of thrombotic microangiopathies can be difficult. Atypical hemolytic uremic syndrome is a rare, life-threatening disease caused by uncontrolled chronic activation of alternative complement pathway, resulting in microvascular thrombosis, organ ischemia and damage. Prognosis is poor: up to 65 percent of patients require dialysis or have kidney damage of varying severity or die despite plasma exchange/plasma infusion treatment.
  
  
  METHODS
  We describe the case of a 23-year-old woman of Hellenic origin who, after a preeclampsia-induced premature delivery, developed thrombotic microangiopathy with renal failure, tonicoclonic seizures, anasarca edema and hypertension. Intensive plasma exchange was initiated twice daily, in parallel to dialysis for one month. Three months later, our patient was discharged with nondialysis-dependent renal failure and without signs of hemolysis. Three months after discharge our patient was readmitted with cardiomyopathy (left ventricular ejection fraction of 25 percent) and signs and symptoms of thrombotic microangiopathy. Our patient was diagnosed with atypical hemolytic uremic syndrome and was started on eculizumab (a complement inhibitor), which improved clinical and laboratory parameters. However, a transient pause in treatment resulted in thrombotic microangiopathy relapse, which was rapidly blocked with reintroduction of eculizumab treatment. During long-term eculizumab treatment, thrombotic microangiopathy manifestations were inhibited and renal and cardiac function restored, with no need for other invasive treatments.
  
  
  CONCLUSIONS
  Establishing the diagnosis of atypical hemolytic uremic syndrome in patients presenting with thrombotic microangiopathy is challenging since common symptoms are shared with other conditions like Shiga toxin-producing Escherichia coli hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. The described case illustrates the complexity and importance of rapid diagnosis in a rare disease and the need for appropriate and specific treatment for best long-term outcomes.
authors.forename: Andreas
authors.initials: A
authors.lastname: Kourouklaris
authors.forename: Kyriakos
authors.initials: K
authors.lastname: Ioannou
authors.forename: Ioannis
authors.initials: I
authors.lastname: Athanasiou
authors.forename: Alexia
authors.initials: A
authors.lastname: Panagidou
authors.forename: Kiproulla
authors.initials: K
authors.lastname: Demetriou
authors.affiliation: Department of Nephrology, Nicosia General Hospital, B1, Strovolos, Nicosia, Cyprus. mzavros@mphs.moh.gov.cy.
authors.forename: Michalis
authors.initials: M
authors.lastname: Zavros
mesh_terms: D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007668:Kidney / Q000473:pathology; D010951:Plasma Exchange / Q000379:methods; D011644:Puerperal Disorders / Q000175:diagnosis / Q000188:drug therapy*; D012008:Recurrence; D006435:Renal Dialysis / Q000379:methods; D057049:Thrombotic Microangiopathies / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab