Figure 1.

The mechanism of action of caplacizumab.

Notes: (A) The pathogenesis of aTTP; the presence of anti-ADAMTS13 autoantibodies inhibits the proteolytic cleavage of ultra-large vWF multimers by ADAMTS13, which results in the aggregation of platelets through GP1b-α receptors and the activated A1 domain of the vWF causing microvascular thrombosis and ischemic organ damage. (B) Caplacizumab blocks the platelet and ultra-large vWF interaction by binding to A1 domain of vWF.

Abbreviations: aTPP, acquired thrombotic thrombocytopenic purpura; GP1b-α, glycoprotein 1b-alpha; vWF, von Willebrand factor.