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Von Willebrand factor (vWF) is produced by cells in the blood vessels and forms large structures. These structures are broken down into smaller units by a protease called ADAMTS13. When ADAMTS13 is not functioning properly, the vWF structures accumulate (It is important to note that ADAMTS13 deficiency can be inherited or acquired through an autoimmune mechanism). Platelets stick to these structures and promote clumping, resulting in clot formation. These micro clots deposit in capillary beds an
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