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Tissue Factor Pathway Inhibitor (TFPI) is a key regulator of the extrinsic coagulation pathway. It inhibits coagulation through the formation of a quaternary complex with Coagulation Factor X, Coagulation Factor III/Tissue Factor, and Coagulation Factor VII, thereby preventing the activation of Factor X. TFPI also exhibits anti-angiogenic and anti-metastatic effects. It is produced by vascular endothelial cells and circulates as a free molecule, on platelet surfaces, and in complex with LDL and HDL particles. Abnormal TFPI concentrations in the circulation are associated with thrombosis, disseminated intravascular coagulation, and sepsis. TFPI can enhance anti-thrombotic treatment in sepsis, inflammatory diseases, and cardiovascular diseases.