Role of lipoprotein apheresis in the management of familial hypercholesterolemia

Abstract

Familial Hypercholesterolemia (FH) is an autosomal dominant disorder due to a congenital absence of low-density lipoprotein receptor (LDL-R) that transports cholesterol-carrying lipoprotein particles into cells. Absence of these receptors results in increased levels Non-HDL lipoproteins (like LDL, VLDL, Triglycerides, Lipoprotein a) which predisposes to premature atherosclerosis and cardiac complications. Treatment modalities for this condition include use of high intensity statins and diet control. However some of these patients with familial hypercholesterolemia do not respond to diet and drug therapy. Here Lipoprotein apheresis is believed to be a feasible and effective option. Lipoprotein apheresis is a relatively new technique in India used to treat familial hypercholesterolemia. Lipoprotein apheresis using cascade filter is effective in reducing non-HDL lipoprotein levels by 60-75% in a single procedure. However, the disadvantage of the procedure it is expensive and needs to be repeated every 2 weeks for lifetime. This case is being reported for rarity of this condition (1 case per million) and more so the rarity of the use of Lipoprotein apheresis procedure in our country.

Keywords

• cascade filtration
• familial hypercholesterolemia
• lipoprotein apheresis
• management of familial hypercholesterolemia