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 TTP includes fever, neurologic abnormalities, thrombocytopenia with purpura, microangiopathic hemolytic anemia, and acute renal injury. However, < 7% of cases have all of these findings present. Testing should include a complete blood count, complete metabolic panel, blood smear, coagulation panel, fibrinogen, D-dimer, lactate dehydrogenase, ADAMTS13 level, troponin, human immunodeficiency virus assessment, urinalysis, pregnancy test as appropriate, and electrocardiogram. Management includes hematology consultation if available, plasma exchange and corticosteroids, and treatment of end-organ complications. All patients require admission for treatment and close monitoring.
Conclusion: 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.
Keywords: TTP; hematology; microangiopathic hemolytic anemia; thrombotic microangiopathy; thrombotic thrombocytopenic purpura.
Published by Elsevier Inc.