#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

Extracorporeal elimination in familial hypercholesterolemia - comparison of two methods


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


Sources

1. Ito MK, McGovan MP, Moriarty, PM. Management of familial hypercholesterolemias in adult patients. Recommendation from National Lipid Association Expert panel on familial hypercholesterolemia. J Clin Lipidol 2011; 5: 538-545.

2. Nordestgaard BG, Chapman MJ, Humphries SE, et al. Familial hypercholesterolemia is underdiagnosed and untreated in the general population: guidance for clinicians to prevent coronary heart disease. Consensus statement of the European Atherosclerosis Society. Eur Heart J 2013; 34(45): 3478-90.

3. Donato LJ, Seanger AK, Train JL, et al. Genetic and biochemical analysis in dyslipidemic patients undergoing LDL apheresis. J Clin Apher; publikováno elektronicky 2014 Jan 13. doi: 10.1002/jca.21317.

4. Hopkins PN, Toth PP, Ballantyne CM, Rader DJ. Familial hypercholesterolemias: prevalence, genetics, diagnosis and screening recommendations from the National Lipid Association Expert Panel on familial hypercholesterolemia.lin Lipidol 2011 Jun; 5(3 Suppl): 9-17.

5. Goldberg AC, Hopkins PN, Toth PP, et al. Familial hypercholesterolemia: screening, diagnosis and management of pediatric and adults patients. J Clin Lipidol 2011; 5: 1-8.

6. Berge KE, Tian H, Graf GA, et al. Accumulation of dietary cholesterol in sitosterolemia caused by mutations in adjacent ABC transporters. Science 2010; 290 (5497): 1771–5.

7. Sniderman AD, Tsimikas S, Fazio S. The severe hypercholesterolemia phenotype. J Am Coll Cardiol 2014; 63(19): 1935-1947.

8. Shib MH, Arai H., Oikawa S, Ohta T, Okada T, Okamura T, et al. Guidelines for the managment of familial hypercholesterolemia. J Atheroscler Thromb, 2012; 19: 1043-1060

9. Vishwanath R, Hemphill LC. Familial hypercholesterolemia and estimation of US patients eligible for low-density lipoprotein apheresis after maximally tolerated lipid-lowering therapy. J Clin Lipidol 2014; 8: 18-28.

10. Versmissen J1, Botden IP, Huijgen R, et al. Maternal inheritance of familial hypercholesterolemia caused by the V408M low-density lipoprotein receptor mutation increases mortality. Atherosclerosis 2011 Dec; 219(2): 690-3. doi: 10.1016/j.atherosclerosis.2011.08.039.

11. Widhalm K, Binder CB, Kreissl A, et al. Sudden death in a 4-year-old boy: a near-complete occlusion of the coronary artery caused by an aggressive low-density lipoprotein receptor mutation (W556R) in homozygous familial hypercholesterolemia. J Pediatr 2011; Jan: 158-167.

12. Nemati MH. Coronary revascularization in a child with homozygous familial hypercholesterolemia. Interact Cardiovasc Thorac Surg 2010 Jan; 10(1): 131-2.

13. Raal FJ, Santos RD. Homozygous familial hypercholesterolemia: current perspectives on diagnosis and treatment. Atherosclerosis 2012; 223: 262-268.

14. Schwarz J, Winters JL, Padmanabhan A. Guidelines on the use of therapeutic apheresis in clinical practice evidence-based approach from the writing committee of the American Society for Apheresis: the sixth special issue. J Clin Apher 2013; 28: 145–284.

15. Borberg HS, Stoffel W, Greve V. Application of specific extracorporeal removal of low density lipoprotein in familial hypercholesterolaemia. Lancet 1981; 7; 2(8254): 1005-1007.

16. Leebman J, Roeseler E, Julius U, Heigl F, Spitthoever R, Heutling D, et al. Lipoprotein apheresis in patients with maximally tolerated lipid-lowering therapy, lipoprotein(a)-hyperliporoteinemia, and progressive cardiovascular disease: prospective observational multicenter study. Circulation 2013; 128: 2567-2576.

17. Bláha M, Rencová E, Malý R, Dršata, J, Blažek M. Léčba hemorheoferézou (současný stav a vlastní zkušenosti). Aktuality z nefrologie, 14, 2008, č. 3, s.118-124.

18. Borberg HS. 26 years of LDL--apheresis: a review of experience. Transfus Apher Sci. 2009 Aug; 41(1): 49-59.

19. Masin V, Blaha M, Stransky P, et al. Optimization of therapeutic procedure during LDL-apheresis - verification of the computerized model in clinical practice. Transfus Apher Sci. 2007 Feb; 36(1): 39-45.

20. Heigl F, Hettich R, Eder B, Arendt R. Lipoprotein apheresis standard for apheresis competence center - an update synthesis and amendment to pro-existing standards. Atherosclerosis Suppl 14 2013; 57-65.

21. Kalinová M, Zadák Z, Bláha M. Léčba hyperlipoproteinémií kontinuálními plazmaferézami. Lék. Zprávy (Hradec Králové) 26, 1981; 7/8: 135-142.

22. Thompson GR, Miller JP, Breslow JL. Improved survival of patients with homozygous familial hypercholesterolaemia treated by plasma exchange. Br Med J 1985; 291: 1674-3.

23. Bláha M, Zadák Z, Havel E, Bláha V, Malý J, Solichová M. Léčba těžké hyperlipoproteinémie LDL-aferézou. Transfuze dnes 1997; 19: 9-10.

24. Thompson GR. The evidence-base for the efficacy of lipoprotein apheresis min combating cardiovascular disease. Atherosclerosis Suppl. 14 2013; 67-70.

25. Watts GF, Gidding S, Wierbicki AS, Toth PP, Alonso R, Brown WV, et al. Integrated guidance on the care of familial hypercholesterolemia from international FH Foundation. J Clin Lipidol 2014; 8: 148-172.

26. Palcoux JB, Meyer M, Jouanel P, Vanlieferinghen P, Malpuech G. Comparison of different treatment regimens in a case of homozygous familial hypercholesterolemia. Ther Apher 2002; 6(2): 136-139.

27. Raal FJ, Pilcher GJ, Panz VR, van Deventer HE, Brice BC, et al. Reduction in Mortality in subjects with homozygous familial hypercholesterolemia associated with advances in lipid-lowering therapy. Circulation 2011; 124: 2202-2207.

28. Julius U, Parthofer KG, Heibges A, Kurz S, Klingel R, Geiss HC. Dextran-sulfate-adsorption of atherosclerotic liporoteins from whole blood of separated plasma for lipid-apheresis-comparison of performance characteristics with DALI and lipidfiltration. J Clin Apheresis 2007; 22: 215-223.

29. Julius U, Frind A, Tselmin S, Kopprasch S, Poberschin I, Siegert G. Comparison of different LDL apheresis methods. Expert Rev Cardiovasc Ther 2008; 6(5): 629-639.

30. Julius U, Fischer S, Schatz U, Passauer J, Bornstein S. Why an apheresis center should offer more than one lipoprotein apheresis method. Therapeutic Apheresis and Dialysis 2013; 17(2): 179-184.

31. Bláha M, Rencová E, Langrová H, et al. The importance of rheological parameters in the therapy of the dry form of age-related macular degeneration with rheohaemapheresis. Clin Hemorheol Microcirc 2012; 50(4): 245-55.

32. Dršata J, Bláha M, Chrobok V, Rencová E, Lánská M. Léčba percepční ztráty sluchu pomocí reohemaferézy“ Přijato do tisku časopisu Otorinolaryngologie a foniatrie 8.10.2012.

33. Marais AD, Blom DJ. Recent advances in the treatment of homozygous familial hypercholesterolemia. Curr Opin Lipidol 2013; 24: 288-294.

Labels
Haematology Internal medicine Clinical oncology

Article was published in

Transfusion and Haematology Today

Issue 3

2014 Issue 3

Most read in this issue
Topics Journals
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#