Thrombotic thrombocytopenic purpura: a hematological emergency.

BACKGROUND: Thrombotic thrombocytopenic purpura is a hematological emergency and diagnostic challenge. The critical determinant of outcome is timely diagnosis and treatment.

OBJECTIVES: Describe the pathophysiology, presentation, diagnosis, and treatment of thrombotic thrombocytopenic purpura.

DISCUSSION: Thrombotic thrombocytopenic purpura has a varied presentation and a tendency to mimic several disorders. However, it may be at least provisionally diagnosed in the patient with thrombocytopenia and microangiopathic hemolytic anemia without alternate cause. The mainstay of treatment is immediate plasma exchange to be repeated until platelet count is stabilized. Adjuvant therapies include corticosteroids, rituximab, and cyclosporine.

CONCLUSION: It is essential for the emergency physician to be aware of thrombotic thrombocytopenic purpura's range of presentations, diagnostic criteria, and treatment.