Extracorporeal elimination in familial hypercholesterolemia - comparison of two methods

Czech version

Authors: M. Lánská; M. Bláha; P. Žák
Authors‘ workplace: IV. Interní hematologická klinika, Univerzita Karlova v Praze, Lékařská fakulta a Fakultní nemocnice Hradec Králové
Published in: Transfuze Hematol. dnes,20, 2014, No. 3, p. 67-75.
Category: Comprehensive Reports, Original Papers, Case Reports

Overview

Introduction:
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.

Patients and Methods:
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.

Results:
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.

Conclusion:
Treatment of FH is very effective when indicated. There is a significant decrease in all observed parameters. No patient experienced worsening of atherosclerosis. Both methods are safe with minimum adverse reactions. Immunoadsorption is more effective in CH elimination. RHF can be used in patients with hyperfibrinogenemia as an additional risk factor of atherosclerosis. Care of these patients is costly and requires an experienced team and an interdisciplinary approach.

Key words:
familial hypercholesterolemia, LDL cholesterol, immunoadsorption, rheohemapheresis

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