Evaluation and Management of Thrombotic Thrombocytopenic Purpura in the Emergency Department

Background: Thrombotic thrombocytopenic purpura (TTP) is a dangerous condition that can be misdiagnosed in the emergency department. Objective: The purpose of this narrative review article is to provide a summary of the background, pathophysiology, diagnosis, and management of TTP, with a focus on emergency clinicians. Discussion: TTP is a disorder with microangiopathic hemolytic anemia, severe thrombocytopenia, and multiorgan ischemic injury. It may be acquired or hereditary, and is caused by a reduced amount or function of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), which is an enzyme involved in cleaving von Willebrand factor. The classic presentation of topenia with purpura, microangiopathic hemolytic anemia, and acute renal injury. However, < 7% of cases have all of these findings present. Testing should include a complete ADAMTS13 level, troponin, human immunodeficiency virus assessment, urinalysis, pregnancy test as appropriate, and sultation if available, plasma exchange and corticosteroids, and treatment of end-organ complications. All patients re quire admission for treatment and close monitoring. Con-clusion: TTP is a potentially dangerous medical condition requiring rapid diagnosis and management. It is essential for emergency clinicians to know how to diagnose and treat this disorder. Published by Elsevier Inc.