Advantages and limits of ADAMTS13 testing in the prognostic assessment of thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare but severe disease characterized by mechanical hemolytic anemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis that causes signs and symptoms of organ ischemia and functional damage. After the elucidation of the pathophysiology of TTP, thanks to the demonstration of the congenital or acquired (autoimmune) plasma deficiency of the von Willebrand factor cleaving metalloprotease A Disintegrin And Metalloprotease with Thrombo. Spondin 1 repeats (ADAMTS13), a number of laboratory assays for measuring ADAMTS13 and related autoantibodies have been developed. Current knowledge on the diagnostic and prognostic value of ADAMTS13 and anti-ADAMTS13 assays is summarized in this review.