Lipoprotein apheresis efficacy, challenges and outcomes: A descriptive analysis from the UK Lipoprotein Apheresis Registry, 1989–2017

Pottle, Alison; Thompson, Gilbert R.; Barbir, Mahmoud; Bayly, Graham; Cegla, Jaimini; Cramb, Robert; Dawson, Tina; Eatough, Ruth; Kale, Vaishali; Neuwirth, Clare; Nicholson, Kirsty; Payne, Jules; Scott, James A.; Soran, Handrean; Walji, S.; Watkins, Suzanne; Weedon, Hazel; Datta, D.

doi:10.1016/j.atherosclerosis.2019.09.006

Cited by 31 publications

(9 citation statements)

References 30 publications

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“…The LDL-C goals achieved in our LA cohort are consistent with those reported in previous studies where LA was used as the sole therapeutic intervention [6,7,18]. Recently published data from the UK Lipoprotein Apheresis Registry including both HeFH (N = 58) and HoFH (N = 30) have shown that the overall reduction in LDL-C interval means was 43.14% [19]. Furthermore, in a previous analysis by Bruckert et al [6], authors found a reduction in TC by 20% in LA treated patients.…”

Section: Discussionsupporting

confidence: 88%

Long-term efficacy of lipoprotein apheresis and lomitapide in the treatment of homozygous familial hypercholesterolemia (HoFH): a cross-national retrospective survey

D’Erasmo

Cefalù

Costanzo

et al. 2021

Orphanet J Rare Dis

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Background Homozygous familial hypercholesterolemia (HoFH) is a rare life-threatening condition that represents a therapeutic challenge. The vast majority of HoFH patients fail to achieve LDL-C targets when treated with the standard protocol, which associates maximally tolerated dose of lipid-lowering medications with lipoprotein apheresis (LA). Lomitapide is an emerging therapy in HoFH, but its place in the treatment algorithm is disputed because a comparison of its long-term efficacy versus LA in reducing LDL-C burden is not available. We assessed changes in long-term LDL-C burden and goals achievement in two independent HoFH patients’ cohorts, one treated with lomitapide in Italy (n = 30) and the other with LA in France (n = 29). Results The two cohorts differed significantly for genotype (p = 0.004), baseline lipid profile (p < 0.001), age of treatment initiation (p < 0.001), occurrence of cardiovascular disease (p = 0.003) as well as follow-up duration (p < 0.001). The adjunct of lomitapide to conventional lipid-lowering therapies determined an additional 58.0% reduction of last visit LDL-C levels, compared to 37.1% when LA was added (padj = 0.004). Yearly on-treatment LDL-C < 70 mg/dl and < 55 mg/dl goals were only achieved in 45.5% and 13.5% of HoFH patients treated with lomitapide. The long-term exposure to LDL-C burden was found to be higher in LA than in Lomitapide cohort (13,236.1 ± 5492.1 vs. 11,656.6 ± 4730.9 mg/dL-year respectively, padj = 0.002). A trend towards fewer total cardiovascular events was observed in the Lomitapide than in the LA cohort. Conclusions In comparison with LA, lomitapide appears to provide a better control of LDL-C in HoFH. Further studies are needed to confirm this data and establish whether this translates into a reduction of cardiovascular risk.

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Section: Discussionsupporting

confidence: 88%

Long-term efficacy of lipoprotein apheresis and lomitapide in the treatment of homozygous familial hypercholesterolemia (HoFH): a cross-national retrospective survey

D’Erasmo

Cefalù

Costanzo

et al. 2021

Orphanet J Rare Dis

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“…Moreover, the clinical characteristics of the GLAR patients were comparable to our study 36 . Another registry, evaluating LA treatment data from the United Kingdom, reported a 62.5% reduction in MACE in a comparable time period 38 . The substantial reduction in ACVE was also observed in the 30 individuals with FH or familial combined hypercholesterolemia and CVD studied by Sampietro et al The incidence rate of ACVE prior to and after LA initiation was 86 and 15 events, respectively.…”

Section: Discussionsupporting

confidence: 83%

“…Moreover, the authors indicated that the clinical success of LA can be related to the removal of numerous atherogenic proteins, inflammatory markers or other apoB‐containing lipoproteins 37 . Another study from the United Kingdom reported the reduction of the interval mean LDL‐C by 43% and that of Lp(a) levels by 38% as the indicators of LA efficacy 38 …”

Section: Discussionmentioning

confidence: 99%

Cardiovascular events in patients with familial hypercholesterolemia and hyperlipoproteinaemia (a): Indications for lipoprotein apheresis in Poland

Mickiewicz

Marlęga

Kuchta

et al. 2021

J of Clinical Apheresis

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Background Lipoprotein apheresis (LA) is a safe method of reducing atherogenic lipoproteins and improving cardiovascular (CV) outcomes. We aimed to assess the reductions in low‐density lipoprotein cholesterol (LDL‐C) and lipoprotein (a) [Lp(a)] levels in patients undergoing regular LA therapy and to evaluate its influence on the incidence rate of adverse cardiac and vascular events (ACVE) and major adverse cardiac events (MACE). Methods A longitudinal study in Poland evaluated the prospective and retrospective observational data of 23 patients with hyperlipoproteinaemia (a) [hyper‐Lp(a)] and familial hypercholesterolemia (FH), undergoing 1014 LA sessions between 2013 and 2020. Their pre‐ and post‐apheresis LDL‐C and Lp(a) levels were assessed to calculate the acute percent reductions. The time period used to evaluate annual rates of ACVE and MACE before and after initiation of LA was matched in each patient. Results The pre‐apheresis LDL‐C and Lp(a) concentrations were 155 (107‐228) (mg/dL) (median and interquartile range) and 0.56 (0.14‐1.37) (g/L), respectively. LA therapy resulted in a reduction of LDL‐C to 50 (30‐73.5) (mg/dL) and of Lp(a) to 0.13 (0.05‐0.34) (g/L), representing a percent reduction of 70.0% and 72.7% for LDL‐C and Lp(a), respectively. We found a significant reduction in the annual rate of ACVE (0.365[0.0‐0.585] vs (0.0[0.0‐0.265]; P = .047) and MACE (0.365[0.0‐0.585] vs 0.0[0.0‐0.265]; P = .031). Conclusions The findings of our study indicate that LA treatment in patients with hyperlipoproteinaemia (a) and FH on maximally tolerated lipid lowering therapies leads to a substantial reduction in LDL‐C and Lp(a) concentrations and lowers CV event rates in Polish patients.

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“…Currently, the most popular methods in the UK are dextran sulfate adsorption and polyacrylate/polyacrylamide adsorption of apoB‐containing lipoproteins from whole blood, and double filtration plasmapheresis. Less popular are dextran sulfate adsorption and heparin‐induced precipitation of apoB‐containing lipoproteins from plasma [4]. The term selective is a misnomer because these procedures remove not only LDL but also Lp(a), very low‐density lipoproteins (VLDL) and chylomicrons, all of which contain apoB.…”

Section: Historical Backgroundmentioning

confidence: 99%

The scientific basis and future of lipoprotein apheresis

Thompson

2021

Ther Apher Dial

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Lipoprotein apheresis plays a vital role in the management of the severe hyperlipidemias that predispose to atherosclerosis. Determinants of efficacy are the acute reduction in lipoproteins achieved by each apheresis procedure, their frequency, and the fractional catabolic rates and hence pool sizes of low‐density lipoprotein (LDL) or lipoprotein (a) (Lp(a)) of the patient being treated. A useful criterion of the efficacy of apheresis plus lipid‐lowering drug therapy is the decrease in the interval (time‐averaged) mean of serum total or LDL cholesterol or Lp(a) between procedures, expressed as the percent decrease in the interval means below the maximal levels of these lipoproteins when off all treatment. Recent advances in lipid‐lowering drug therapy may diminish the use of lipoprotein apheresis but will not abolish its unique role as a therapeutic “last chance saloon,” especially for children and pregnant women with homozygous familial hypercholesterolemia.

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Lipoprotein apheresis efficacy, challenges and outcomes: A descriptive analysis from the UK Lipoprotein Apheresis Registry, 1989–2017

Cited by 31 publications

References 30 publications

Long-term efficacy of lipoprotein apheresis and lomitapide in the treatment of homozygous familial hypercholesterolemia (HoFH): a cross-national retrospective survey

Long-term efficacy of lipoprotein apheresis and lomitapide in the treatment of homozygous familial hypercholesterolemia (HoFH): a cross-national retrospective survey

Cardiovascular events in patients with familial hypercholesterolemia and hyperlipoproteinaemia (a): Indications for lipoprotein apheresis in Poland

The scientific basis and future of lipoprotein apheresis

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