Plasminogen Activator Inhibitor-1 deficiency

Gulf States Hemophilia Center iconPlasminogen activator inhibitor-1 (PAI-1) deficiency is a rare inherited autosomal recessive bleeding disorder characterized by excessive clot lysis leading to a lifelong moderate bleeding diathesis. PAI-1 is an essential protein critical in down-regulation of the fibrinolytic pathway.

Decreased PAI-1 activity may be caused by qualitative or quantitative defects. PAI-1 deficiency is a mild to moderate delayed bleeding disorder. The most common bleeding symptoms include those following surgery, injury, or trauma and in women include menorrhagia, rarely hemorrhagic rupture of ovarian cysts, antenatal bleeding and post-partum hemorrhage.