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ADAMTS13: A Disintegrin-likeAnd Metalloprotease with ThromboSpondin type 1 repeats is a von Willebrandfactor-cleaving metalloproteinase. The level of ADAMTS13 in normal plasma isestimated 900 ng/ml. Deficiency of ADAMTS13 results in the development ofclinical entity Thrombotic Thrombocytopenic Purpura (TTP) and may play a rolein Hemolytic Uremic Syndrome. Von Willebrand’s Factor is synthesized byendothelial cells and stored in the sub-endothelial space as very large multimericstructures. These ULVWf avidly bind platelets when they are exposed in thesetting of endothelial disruption and lead to platelet clumping and closureof the endothelial rent. Any ULVWf that escapes into circulation is rapidlydegraded by ADAMTS13. Deficiency of ADAMTS13 (congenital or acquired) leadsto the accumulation of ULVWf in the plasma with resultant systemic plateletaggregation and clumping. This leads to the clinical syndrome of TTPcharacterized by thrombocytopenia, microangiopathic hemolytic anemia,neurologic deficits, renal abnormalities and fever.
Congenitaldeficiency of ADAMTS13 (Upshaw-Schulman Syndrome) is characterized byADAMTS13 enzymatic activity of 2-7% or normal. Modest reductions in ADAMTS13have been noted in a variety of conditions, such as severe sepsis, disseminatedintravascular coagulation, heparin-induced thrombocytopenia, cardiac surgeryand cirrhosis.