Thrombotic microangiopathy mediated by complement in postpartum sequentially treated With plasmapheresis and eculizumab

Abstract

The complement-mediated thrombotic microangiopathy (TMA), also known as atypical hemolytic uremic syndrome (aHUS), is rare and potentially fatal, presenting as TMA and dysfunction/endothe - lial edema secondary to diffuse disruption of a fluid phase alternat - ing complementary pathways. We report a case of a young woman with postpartum anuric renal failure, TMA and normal levels of AD- AMTS13 with partial clinical improvement after plasmapheresis, in which treatment with eculizumab led to the complete reversal of the clinical manifestations. Our work includes a brief literature review on the diagnosis and management of TMA in puerperium. mor cells; small vessel vasculitis, autoimmune or infectious etiology; endothelial dysfunction, which may be mediated by complement or Shiga-like toxin [1]. The period of pregnancy and postpartum eventually com- plicates these conditions, by immunological changes (predis- posing the formation of autoantibodies), changes in clotting factors with decreased level of ADAMST13 enzyme, which cleaves factor multimers of von Willebrand, and own pregnan- cy (preeclampsia and clinical spectra). Most of the time, the differential diagnosis of these diseases is difficult, requiring a multidisciplinary approach for the best outcome of the patient. We report a case of a young postpartum, with hypertensive disorders of pregnancy, with TMA initially conducted as TTP, with partial response to plasmapheresis and complete response to eculizumab.