Control of bleeding is an essential requirement of surgery, and the risks of excessive bleeding are of major concern to surgeons. This is especially true for liver surgeons in view of the coagulopathy associated with the lack of essential clotting factors in patients with decompensated liver disease. However, it would now appear that the lack of certain factors produced by the liver may, in fact, also render the patient hypercoagulable.
The role of ADAMTS13 (a disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13) in hemostasis has been extensively documented in the hematology literature. In contrast, ADAMTS13 has hardly been noticed on the surgical radar. ADAMTS13 is a specific metalloproteinase that is responsible for cleaving the multimeric form of von Willebrand factor, thus preventing platelet aggregation and thrombus formation.1 Conversely, in the absence of ADAMTS13 activity, or with a profound deficiency of ADAMTS13, due to either genetic mutations or autoimmune inhibitors, ultralarge von Willebrand factor produced by vascular endothelial cells accumulate and induce widespread microvascular thrombosis characteristic of thrombotic thrombocytopenic purpura.