Figure 1.

Systemic inflammation results in systemic coagulation. Thrombotic thrombocytopenuc purpura (TTP) is a microangiopathy phenotype characterized by ADAMTS 13 deficiency. Left: Platelets attach to ultra large vWF multimers. Because vWF-CP (ADAMTS 13) is inhibited this leads to massive vWF:platelet thrombosis (right). Ab, antibody; CP, cleaving protease; vWF, von Willebrand factor.