1. Figure 1 Mechanism of action of caplacizumab
Figure 1 | Mechanism of action of caplacizumab. a | In patients with acquired thrombotic thrombocytopenic purpura (TTP), platelets adhere via glycoprotein Ibα to activated A1 domains of ultra-large von Willebrand factor (VWF) multimers that are attached to the endothelial cells and stretched under high fluid shear stress in the microcirculation. Adherent platelets bind circulating ultra-large VWF multimers leading to platelet clumping and the development of microvascular thrombosis. b | Caplacizumab binds to the A1 domains of VWF preventing platelet adhesion and microvascular thrombosis and enabling normalization of the platelet count.
Lämmle, B. (2016) Caplacizumab accelerates resolution of acute acquired TTP
Nat. Rev. Nephrol. doi: /nrneph