Lipoprotein apheresis in the treatment of dyslipidaemia - the Czech Republic experience

Area of Science:

• Cardiology and Vascular Medicine
• Biochemistry and Metabolism

Background:

• Familial hypercholesterolemia (FH) is a severe genetic disorder characterized by extremely high LDL-cholesterol levels.
• Traditional lipid-lowering therapies are often insufficient for homozygous FH patients, necessitating alternative treatments.
• Extracorporeal lipoprotein elimination methods have been explored for managing severe hypercholesterolemia since 1984.

Purpose of the Study:

• To evaluate the long-term efficacy and safety of therapeutic plasmapheresis, specifically lipoprotein apheresis and hemorheopheresis, in patients with severe hypercholesterolemia.
• To assess the impact of these apheresis techniques on lipid profiles, atherosclerosis markers, and microcirculation.
• To determine the overall tolerance and complication rates associated with long-term apheresis therapy in FH patients.

Main Methods:

• Long-term therapeutic plasmapheresis was performed on 14 patients (8 men, 6 women; average age 55.6 years) diagnosed with familial hypercholesterolemia (5 homozygous, 9 heterozygous).
• Ten patients received immunoadsorption lipoprotein apheresis, while four underwent hemorheopheresis.
• Lipid levels (LDL-cholesterol, ApoB, Lp(a)), carotid intima-media thickness, and biomarkers of endothelial dysfunction (endoglin, CD40 ligand, neopterin) were monitored. Hemorheopheresis effects on thrombomodulin, tissue factor, platelet aggregation, and viscosity were also assessed.

Main Results:

• Lipoprotein apheresis significantly reduced LDL-cholesterol by 82±1%, ApoB by 73±13%, and Lp(a) by 82±19%.
• Carotid intima-media thickness improved or stabilized in 75% of patients.
• Hemorheopheresis demonstrated significant reductions in thrombomodulin activity, tissue factor activity, platelet aggregation, and viscosity (p<0.0001), suggesting improved microcirculation. Biomarkers of endothelial dysfunction did not consistently reflect disease activity.
• Long-term therapy showed good tolerance with a low rate of non-serious clinical complications (6.26%).

Conclusions:

• Lipoprotein apheresis and hemorheopheresis are effective in substantially lowering LDL-cholesterol in severe hypercholesterolemia, including familial hypercholesterolemia.
• These extracorporeal methods are well-tolerated and associated with a low incidence of complications during long-term use.
• Hemorheopheresis may offer additional benefits by improving microcirculation, while certain endothelial dysfunction biomarkers require further investigation for therapeutic relevance.