Understanding the Clotting Cascade, Regulators, and Clinical Modulators of Coagulation

Abstract

The circulatory system plays a vital role in the survival of an organism by supplying it with essential nutrients, signaling molecules and eliminating the waste or toxic products from the body. This flow is tightly regulated by various factors, procoagulants support the formation of hemostatic plugs to prevent the leakage or blood loss and anticoagulants prevent the formation of unwanted clots. Disruption or dysregulation of procoagulants and anticoagulants lead to clinical complexities. In this chapter defects in the coagulation system, hereditary, acquired coagulation disorders, their diagnosis and recent clinical modulators of the coagulation system are discussed.

Current therapeutic approaches in the management of hemophilia—a consensus view by the Romanian Society of Hematology

“…During an injury or a surgical intervention, the prevention of massive blood loss requires immediate actions to be taken, while maintaining blood fluidity and restricting the clotting process only to the site of vascular injury (prevention of thrombosis). Therefore, a complex and dynamic therapeutic approach, which includes an equilibrium of procoagulants, anticoagulants, and fibrinolytic drugs enable hemostasis without causing thrombosis (1). These drugs include serine protease zymogens and protein cofactors activated in a certain order and initiate the coagulation cascade at the site of vascular damage (2)(3)(4).…”

“…Hemophilia A (HA) and hemophilia B (HB) are two rare genetic disorders (1), caused by the total lack or the under-expression of two factors from the coagulation cascade coded by genes of the X chromosome (2). Thus, in hemophilic patients the blood does not clot properly.…”