open access

Vol 26, No 6 (2019)
Original articles — Clinical cardiology
Published online: 2018-09-07
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Long-term lipoprotein apheresis in the treatment of severe familial hypercholesterolemia refractory to high intensity statin therapy: Three year experience at a lipoprotein apheresis centre

Agnieszka Mickiewicz, Justyna Borowiec-Wolna, Witold Bachorski, Natasza Gilis-Malinowska, Rafał Gałąska, Grzegorz Raczak, Magdalena Chmara, Bartosz Wasąg, Miłosz J. Jaguszewski, Marcin Fijałkowski, Marcin Gruchała
DOI: 10.5603/CJ.a2018.0100
·
Pubmed: 30234904
·
Cardiol J 2019;26(6):669-679.

open access

Vol 26, No 6 (2019)
Original articles — Clinical cardiology
Published online: 2018-09-07

Abstract

Background: Severe familial hypercholesterolemia (FH) individuals, refractory to conventional lipidlowering
medications are at exceptionally high risk of cardiovascular events. The established therapeutic
option of last choice is lipoprotein apheresis (LA). Herein, it was sought to investigate the clinical usefulness
of LA in a highly selected group of severe heterozygous FH (HeFH), as recently described by the
International Atherosclerosis Society (IAS), for their efficacy in lipid reduction and safety.
Methods: Efficacy and safety of LA were investigated in 318 sessions of 7 severe HeFH females with
cardiovascular disease, over a mean period of 26.9 ± 6.5 months. Relative reduction of low density lipoprotein
cholesterol (LDL-C) ≥ 60%, clinical complications and vascular access problems were evaluated
and compared between the direct adsorption of lipoproteins (DALI) and lipoprotein filtration (Membrane
Filtration Optimized Novel Extracorporeal Treatment [MONET]). Additionally, lipoprotein (a)
[Lp(a)], total cholesterol (TC), high density lipoprotein cholesterol (HDL-C), triglycerides (TG) and
fibrinogen concentrations were investigated.
Results: The relative reduction of LDL-C, TC, TG and Lp(a) were 69.4 ± 12.9%, 59.7 ± 9.1, 51.5 ±
± 14.2% and 71.3 ± 14.4%, respectively. A similar efficacy was found in both systems in LDL-C removal.
DALI system led to larger depletions of Lp(a) (80.0 [76–83]% vs. 73.0 [64.7–78.8]%; p < 0.001).
The frequency of clinical side effects and vascular access problems were low (8.5%).
Conclusions: Long-term LA in severe HeFH individuals is safe and efficiently reduces LDL-C and
Lp(a). Higher efficacy of the DALI system than MONET in Lp(a) removal may indicate the need for individualized
application of the LA system in severe HeFH individuals.

Abstract

Background: Severe familial hypercholesterolemia (FH) individuals, refractory to conventional lipidlowering
medications are at exceptionally high risk of cardiovascular events. The established therapeutic
option of last choice is lipoprotein apheresis (LA). Herein, it was sought to investigate the clinical usefulness
of LA in a highly selected group of severe heterozygous FH (HeFH), as recently described by the
International Atherosclerosis Society (IAS), for their efficacy in lipid reduction and safety.
Methods: Efficacy and safety of LA were investigated in 318 sessions of 7 severe HeFH females with
cardiovascular disease, over a mean period of 26.9 ± 6.5 months. Relative reduction of low density lipoprotein
cholesterol (LDL-C) ≥ 60%, clinical complications and vascular access problems were evaluated
and compared between the direct adsorption of lipoproteins (DALI) and lipoprotein filtration (Membrane
Filtration Optimized Novel Extracorporeal Treatment [MONET]). Additionally, lipoprotein (a)
[Lp(a)], total cholesterol (TC), high density lipoprotein cholesterol (HDL-C), triglycerides (TG) and
fibrinogen concentrations were investigated.
Results: The relative reduction of LDL-C, TC, TG and Lp(a) were 69.4 ± 12.9%, 59.7 ± 9.1, 51.5 ±
± 14.2% and 71.3 ± 14.4%, respectively. A similar efficacy was found in both systems in LDL-C removal.
DALI system led to larger depletions of Lp(a) (80.0 [76–83]% vs. 73.0 [64.7–78.8]%; p < 0.001).
The frequency of clinical side effects and vascular access problems were low (8.5%).
Conclusions: Long-term LA in severe HeFH individuals is safe and efficiently reduces LDL-C and
Lp(a). Higher efficacy of the DALI system than MONET in Lp(a) removal may indicate the need for individualized
application of the LA system in severe HeFH individuals.

Get Citation

Keywords

lipoprotein apheresis, severe familial hypercholesterolemia, lipoprotein (a)

About this article
Title

Long-term lipoprotein apheresis in the treatment of severe familial hypercholesterolemia refractory to high intensity statin therapy: Three year experience at a lipoprotein apheresis centre

Journal

Cardiology Journal

Issue

Vol 26, No 6 (2019)

Pages

669-679

Published online

2018-09-07

DOI

10.5603/CJ.a2018.0100

Pubmed

30234904

Bibliographic record

Cardiol J 2019;26(6):669-679.

Keywords

lipoprotein apheresis
severe familial hypercholesterolemia
lipoprotein (a)

Authors

Agnieszka Mickiewicz
Justyna Borowiec-Wolna
Witold Bachorski
Natasza Gilis-Malinowska
Rafał Gałąska
Grzegorz Raczak
Magdalena Chmara
Bartosz Wasąg
Miłosz J. Jaguszewski
Marcin Fijałkowski
Marcin Gruchała

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