Figure 3.

Secondary thrombotic microangiopathy (TMA) has a phenotype characterized by decreased ADAMTS 13, and increased plasminogen activator inhibitor type I (PAI-1) and von Willebrand factor (vWF) levels with normal or high fibrinogen levels. Platelets attach to increased large vWF multimers and form thrombi in the presence of decreased PAI-I activity (left), leading to systemic platelet thrombi with delayed fibrinolysis (right). CP, cleaving protease; TF, tissue factor; TFPI, tissue factor pathway inhibitor; vWF-CP, ADAMTS 13.