Thrombotic thrombocytopenic purpura: A review

Since the original description of thrombotic thrombocytopenic purpura (TTP) in 1924, remarkable advances have been made in understanding the pathophysiology and molecular biology of the disease. The description of the structure of "A Distintegrin And Metalloprotease with ThromboSpondin type 1 motif", also known as ADAMTS13, has yielded insight into its role in regulating the multimeric structure of the von Willebrand factor in plasma. Thus, a deficiency of ADAMTS13, either congenital due to gene defect or acquired due to autoantibody, leads to TTP. Therapeutic plasma exchange, originally an empiric attempt to treat TTP, has evolved into the gold standard of treatment supported by evidence-based medicine. This article reviews the advancements in the pathophysiology of TTP that have increased diagnostic accuracy and improved its management.