The knowledge of tissue factor pathway inhibitor (TFPI) has greatly ex
panded during the last few years, and TFPI is now established as a coa
gulation inhibitor of great importance. Its main role seems to be inhi
bition of small amounts of tissue factor which probably is essential f
or maintaining a normal hemostatic balance. The acceleration of TFPl's
inhibitory effect by heparin, the TFPI release caused by heparin inje
ction, and TFPl's heparin affinity may greatly contribute to the antic
oagulant properties of the endothelium, and may be particularly import
ant for the outcome of vascular injury. The information provided by on
e single measurement of plasma TFPI in a patient is difficult to inter
pret, but serial measurements during the course of a disease may signa
l the prognosis. Recombinant TFPI has proved effective in the treatmen
t of experimental disseminated intravascular coagulation sepsis, and t
hrombosis. Whether TFPI or TFPI derivatives will be as effective in th
e treatment of patients remains to be determined.