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Severe ADAMTS13 Deficiency

Severe ADAMTS13 Deficiency

ADAMTS13 is an enzyme produced by the vascular endothelium that cleaves von Willebrand Factor, a protein that mediates platelet adhesion and aggregation at sites of vascular injury. Deficient levels of ADAMTS13 allows platelets to bind to uncleaved von Willebrand Factor multimers and leads to thrombotic microangiopathy.