Eculizumab, a successful treatment for atypical postpartum hemolytic-uremic syndrome: a case report / Eculizumab, un tratamiento exitoso en el síndrome hemolítico-urémico atípico puerperal: un caso clínico

ABSTRACT

Atypical hemolytic-uremic syndrome (aHUS) is a rare entity characterized by the association of acute kidney failure, thrombocytopenia and microangiopathic hemolytic anemia due to the dysregulation of the alternative pathway of the complement system. It is included within the thrombotic microangiopathies. The following aHUS was developed in the immediate puerperium in the context of severe preeclampsia. The patient was a primiparous woman of 30+1 weeks who required hospitalization for anticonvulsant and hypotensive treatment, and who underwent an emergency cesarean section due to a pathological cardiotocographic pattern. 36 hours after delivery, the patient presented with sudden dyspnea and cognitive deterioration, progressing in a few hours to renal and multiorgan failure. Blood test showed severe anemia, thrombopenia and hypertransaminemia. In view of the fast evolution and severity, it was decided to treat with Eculizumab, although the scientific evidence was very poor. Aside from the supportive treatment performed in the Intensive Care Unit, the patient was successfully treated with Eculizumab, with favorable evolution over the following months and restoration of kidney function, although need for chronic hypotensive treatment remained.