Pregnancy-Induced Atypical Hemolytic Uremic Syndrome: Case Report and Review of the Literature [1T]

INTRODUCTION: Pregnancy-induced atypical hemolytic uremic syndrome (p-aHUS), a disease caused by hyperactivation of the alternative complement pathway, can lead to renal ischemia and ESRD. We present two cases of p-aHUS and discuss both the disease process and treatment options. METHODS: The first case, a 31 yo female, underwent a cesarean delivery at 29 weeks due to preterm labor with breech presentation. She developed elevated BP, thrombocytopenia, and elevated serum creatinine postpartum. A nephrology consult confirmed the diagnosis of p-aHUS with elevated LDH and normal ADAMST13 levels. The second case is a 32 yo who underwent a suction D&C due to retained placenta after delivery of an IUFD. Laboratory values revealed thrombocytopenia and acute kidney injury (AKI). Again, Nephrology diagnosed p-aHUS. Both patients underwent plasmapheresis and Eculizumab therapy as soon as p-aHUS was diagnosed. Literature review for p-aHUS revealed the following conclusions: 1) risk factors include postpartum period, history of a cesarean delivery, and a genetic mutation in the CFH gene; 2) other causes of thrombocytopenia, AKI, and hemolytic anemia in pregnancy include pre-E and TTP 3) TTP can be ruled out with a normal ADAMST13 and pre-E can be ruled out by an LDH <1000; and 4) immediate diagnosis and treatment helps to prevent kidney injury. CONCLUSION: Given the rarity of the disease, there is little research involved in p-aHUS. However it is it is important for clinicians to have a strong suspicion for p-aHUS and to initiate treatment immediately to decrease morbidity including ESRD.