Extracorporal Plasma Therapy in the Treatment of Severe Hyper-β-Lipoproteinemia

Abstract

A large and convincing body of evidence links increased coronary risk with both elevated plasma levels of low density lipoprotein (LDL) cholesterol and fibrinogen. Cholesterol in atherosclerotic lesions originates from cholesterol circulating in the blood bound to LDL. Most forms of hyper-β-lipoproteinemia result form a defect in extraction of LDL from plasma by the liver and the LDL-receptor is now being recognized as the crucial element in the control of cholesterol homeostasis. Elevated levels of fibrinogen, a common phenomenon in hypercholesterolemia increases the viscosity of the blood and thereby further alters perfusion of tissues in severe atherosclerotic disease. Furthermore, fibrinogen and its degradation products can both influence prostaglandin metabolism by inhibiting PGI₂ synthesis by endothelial and vascular smooth muscle cells, thereby facilitating platelet aggregation and can also cause injury to endothelial cells.