Thrombotic thrombocytopenic purpura: aetiology, pathophysiology and treatment.

Thrombotic thrombocytopenic purpura (TTP) is a rare disorder whose varied clinical manifestations result from the formation of platelet-rich thrombi within the microvasculature and consequent tissue ischaemia. This review will outline how, in the eighty years since its initial description, scientific discoveries have not only led to a deeper understanding of the fundamental pathophysiology of TTP, but have also contributed to advances in the clinical management of this condition. Current research in this field will hopefully provide the basis for the design and development of novel therapeutic strategies.