title: [Efficacy of eculizumab in a case of pregnancy-associated aHUS].
abstract: Pregnancy-associated thrombotic microangiopathy (TMA) is a rare condition, but it is burdened by a significant perinatal and maternal morbidity as well as mortality. We describe the case of a 33-year-old woman, who developed a TMA at the 36th week of gestation characterized by increased LDH, haptoglobin consumption, schistocytes, thrombocytopenia and acute renal failure requiring dialysis. There were not gestational hypertension nor proteinuria until the day of hospitalization. ADAMTS 13 deficiency was ruled out and the patient did not have diarrhea. She was initially treated with caesarean section, plasma infusion and plasmapheresis with no benefit. Five days after the onset of TMA, a temptative diagnosis of atypical uremic syndrome (aHUS) was made and the patient was switched to eculizumab. Antibiotic prophylaxis and anti-meningococcal A,B, C, W135 and Y vaccination was performed. TMA rapidly resolved and renal function completely recovered. The newborn had a normal perinatal course. A complement dysregulation was ruled out by testing for mutations on CFH, CFHR3-R1, CFI, MCP, CFB, C3 and for anti CFH antibodies. In conclusion the differential diagnosis of aHUS with HELLP syndrome is often not straightforward. The severity and persistence of TMA, the high mortality associated to peripartum TMA and the risk for irreversible kidney failure require an early therapeutic decision as to the use of eculizumab.
authors.forename: Raffaella
authors.initials: R
authors.lastname: Cravero
authors.forename: Gianluigi
authors.initials: G
authors.lastname: Ardissino
authors.forename: Umberto
authors.initials: U
authors.lastname: Colageo
authors.forename: Piernicola
authors.initials: P
authors.lastname: Staffa
authors.forename: Elena
authors.initials: E
authors.lastname: Bruschetta
authors.forename: Serena
authors.initials: S
authors.lastname: Maroni
authors.forename: Eirini
authors.initials: E
authors.lastname: Karvela
authors.forename: Ermanno
authors.initials: E
authors.lastname: Spagarino
authors.forename: Velia
authors.initials: V
authors.lastname: Ruggeri
authors.forename: Roberto
authors.initials: R
authors.lastname: Jura
mesh_terms: D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis*; D006801:Humans; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis / Q000178:diet therapy* / Q000188:drug therapy*; D057049:Thrombotic Microangiopathies / Q000175:diagnosis* / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab