ADAMTS-13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 13) is an enzyme (vWF-cleaving protease or vWF-CP) that specifically cleaves von Willebrand Factor (vWF) multimers, a large protein involved in blood clotting. It is secreted in blood and degrades large vWF multimers. If the activity of ADAMTS-13 is decreased, unusually large vWF multimers may accumulate within blood causing thrombosis due to platelet aggregation, which in turn may lead to TTP (thrombotic thrombocytopenic purpura). Differentiation of TTP from other thrombotic microangiopathies (TMA) is challenging. Read more…