Abstract
Familial hypercholesterolemia is a hereditary disorder with autosomal dominant heritance. The underlying cause involves the gene for the LDL-receptor leading to accelerated atherosclerosis.
Extracorporeal elimination of cholesterol (CH) is indicated in 3-5% of patients not responding to conservative treatment or in homozygous FH. Two methods are used at our institution: immunoadsorption of LDL- cholesterol and rheohemapheresis.
We currently have long term follow-up available for 14 patients with familial hypercholesterolemia (8 males, 6 females) aged 28-70 years (median 57 years). 10 patients are treated with immunoadsorption (5 homozygous and 5 heterozygous) and 4 patients are treated with rheohemapheresis (2 males, 2 females). Median follow-up is 8.5 years.
During immunoadsorption, plasma is collected by continuous separation and flows through alternating pairs of adsorbers in an automatic adsorbing-desorbing device. In rheohemapheresis, plasma is collected similarly but goes through a "second step" - filter.
Procedures are repeated every 2-4 weeks. Cholesterol and LDL- cholesterol values are measured before and after each procedure.1922 procedures have been performed (immunoadsorption 1590 times, rheohemapheresis 332 times).
Average cholesterol and LDL- cholesterol values before the procedure were 5.34 and 3.12 mmol/l in immunoadsorption, 5.07 and 2.86 in rheohemapharesis; after the procedure: 1.73 and 0.72 (a fall of 72% and 85%), resp. 1.96 and 0.97 mmol/l (a drop of 61% and 66% drop). Fibrinogen fell by 22% (from 3.05 to 2.42 g/l) and 64% (from 3.48 to 1.2g/l).
There were 3.1% of adverse reactions and no difference was observed between the two methods.