Thrombotic thrombocytopenic purpura (TTP) refers to the disorder of widespread microvascular thrombosis involving the capillaries and arterioles of the brain and other organs. The incidence of TTP is about 4-5 cases per million people per year. TTP has long been recognized as a complex and life-threatening disease. Without treatment, TTP undertakes a rapid course of deterioration. Plasma exchange or infusion is the mainstay of treatment for TTP. In recent years, our understanding of the basic biochemistry of the VWFADAMTS13 axis has provided valuable insights into the pathogenesis of TTP, as well as the investigation and development of new therapeutic strategies. Although the mortality associated with TTP has been appreciably reduced, much yet remains to be learned to more effectively treat and better understand this disease. The authors take the opportunity to present the case report of a young female with varied signs and symptoms,diagnosed as TTP. The rapidly progressive and fatal course of the acute illness explains the need for considering TTP as a probable diagnosis in ED in a patient with fever with purpura and thrombocytopenia.

Keywords: TTP; Adamts-13; VWF; Coagulopathy.