LDL-apheresis therapy

Abstract

Low-density lipoprotein (LDL)-apheresis is an effective means for acutely lowering plasma cholesterol (LDL reduction > 60%) and reducing cardiovascular events in patients with uncontrolled hypercholesterolemia who are genetically predisposed or resistant to standard therapy. Despite its known benefit, apheresis is performed in only a minority of qualified patients. In addition to reducing plasma cholesterol, apheresis can alter other markers of vascular disease (inflammation, rheology, thrombosis, and fibrinolysis). The acute and chronic changes to the vascular system may warrant LDL-apheresis use in other diseases.

Familial Hypercholesterolemias: Prevalence, genetics, diagnosis and screening recommendations from the National Lipid Association Expert Panel on Familial Hypercholesterolemia

“…20 Heterozygous FH occurs with a frequency of about 1 in every 300 to 500 people and is therefore one of the most commonly occurring congenital metabolic disorders. 2,21,22 Based on this estimate there are approximately 10 million people with FH worldwide. There are some populations with a much higher prevalence of FH, perhaps as high as 1 in 50 to 100 in communities with a 'founder gene', (partial or complete) is only indicative of FH if present under age 45.…”

“…Patients with homozygous FH typically develop CHD by the second decade of life 26 , but death may occur in the first years of life from severe CHD. 22 The genetics of Familial Hypercholesterolemias FH is most commonly attributable to mutations (including deletion, missense, nonsense, and insertion types) in the LDLR gene, resulting in LDL receptors having functional reductions (partial to complete) in the capacity to clear LDL cholesterol from the circulation. Patients can be receptor negative, expressing little to no LDL receptor activity, or receptor defective, leading to the expression of LDLR isotypes with reduced affinity for LDL on the hepatocyte surface.…”

Pediatric aspects of Familial Hypercholesterolemias: Recommendations from the National Lipid Association Expert Panel on Familial Hypercholesterolemia

“…25 However, the majority will require LDL apheresis, which is an FDA-approved process of selectively removing Apo B-containing particles from the circulation through extracorporeal precipitation. 74 The procedure is performed at medical centers with this expertise and must be repeated every 1 to 2 weeks. A listing of sites qualified to perform LDL apheresis is in development and will be posted on the National Lipid Association website (www.lipid.org).…”

Management of Familial Hypercholesterolemias in adult patients: Recommendations from the National Lipid Association Expert Panel on Familial Hypercholesterolemia

“…[48][49][50][51] The procedure must be repeated every 1 to 2 weeks. In a single procedure, LDL apheresis typically removes at least 60% of the Apo B-containing lipoproteins.…”