Suspected pregnancy-associated atypical hemolytic uremic syndrome treated with eculizumab: a case report

Abstract

We report a case of suspected pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) successfully treated with eculizumab. A 24-year-old female was transferred to our hospital following a cesarean section and exhibited thrombocytopenia, hemolytic anemia, and renal dysfunction. Thrombotic thrombocytopenic purpura (TTP) or pregnancyassociated atypical hemolytic uremic syndrome (P-aHUS) was suspected. A 2-day consecutive plasma exchange therapy was initiated from the second day of admission. Based on the postpartum onset, absence of CNS symptoms, and severe renal dysfunction, there was a higher likelihood of p-aHUS than TTP, so anti-complement therapy (eculizumab 900 mg/week) was initiated on the third day. The platelet count increased from the fourth day, and serum Cr levels decreased from the fifth day. Thereafter, both the platelet count and Cr levels improved steadily. The activity of a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs number 13 (ADAMTS13) was found normal on the seventh day, and TTP was ruled out. When aHUS is clinically suspected, immediate implementation of anti-complement therapy must be considered while awaiting a final differential diagnosis.