Background: Background:Acquired Thrombotic Thrombocytopenic Purpura (aPTT) is a rare disease caused by the action of antibodies against ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), with high mortality when not treated properly. Formerly, it was defined by the pentad of fever, microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms and severe acute kidney injury. Currently, it is characterized by the presence of microangiopathic hemolytic anemia, thrombocytopenia and variable target organ lesion. Despite its rarity, a prompt diagnosis is essential and the overlapping symptoms with other thrombotic microangiopathies, as well as the multiplicity of nonspecific manifestations, makes its diagnosis highly challenging.
Thrombotic microangiopathy is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ injury. Pregnancy-associated thrombotic microangiopathy is a severe disorder with a high risk of maternal mortality and poor fetal outcomes. Preeclampsia/eclampsia and hemolysis, elevated liver enzymes, and low platelets syndrome are the most common causes, and hemolytic uremic syndrome or thrombotic thrombocytopenic purpura are rare causes. Due to overlapping clinical findings, the differential diagnosis is challenging and should be managed by a multidisciplinary team. We present a case of a 38-year-old woman at 40 weeks of second gestation, admitted with thrombotic microangiopathy being the final diagnosis not immediately clear.
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