Pb2309: Evaluation of Morbimortality Factors of Patients With Acquired Thrombotic Thrombocytopenic Purpura

Abstract

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.