Rapid normalization of severe hypercholesterolemia mediated by lipoprotein X after liver transplantation in a patient with cholestasis - a case report

• Authors: Krzysztof Jankowski , Anna Wyzgał , Aldona Wierzbicka , Olga Tronina , Magdalena Durlik , Piotr Pruszczyk
• Languages of publication: EN

Abstracts

EN

Hypercholesterolemia is a common disorder in adult population, but total cholesterol concentrations beyond 1000 mg/dl occur rarely, and are found in patients with homozygous familial hypercholesterolemia and familial lecithin-cholesterol acyltransferase deficiency, in chronic graft-versus-host disease of the liver, after intravenous infusion of fat emulsion (intralipid), in newborn infants with immature liver function, and in obstructive biliary cholestasis. Cholestasis induces a dramatic increase in plasma cholesterol and the appearance of an abnormal lipoprotein, lipoprotein X (LpX), in the plasma. We report a case of severe hypercholesterolemia mediated by LpX in a patient transplanted for primary biliary cirrhosis (PBC), who was qualified for liver re-transplantation (re-LTx) due to chronic cholestasis. Four months after re-LTx, the cholesterol concentration was normal. The problems in diagnosis and treatment are discussed.

Keywords

EN

hypercholesterolemia; lipoprotein X; PBC

• Publisher: Polish Biochemical Society
• Journal: Acta Biochimica Polonica
• Year: 2015
• Volume: 62
• Issue: 3
• Pages: 621-623

Dates

published

2015

received

2015-01-10

revised

2015-05-24

accepted

2015-07-31

(unknown)

2015-08-28

Contributors

author

Krzysztof Jankowski

• Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland

author

Anna Wyzgał

• Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland

author

Aldona Wierzbicka

• Department of Biochemistry and Experimental Medicine, Children's Memorial Health Institute, Warsaw, Poland

author

Olga Tronina

• Department of Transplant Medicine and Nephrology, Medical University of Warsaw, Poland

author

Magdalena Durlik

• Department of Transplant Medicine and Nephrology, Medical University of Warsaw, Poland

author

Piotr Pruszczyk

• Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland

References

• Catapano AL, Reiner Z, De Backer G, Graham I, Taskinen MR, Wiklund O, Agewall S, Alegria E, Chapman M, Durrington P, Erdine S, Halcox J, Hobbs R, Kjekshus J, Filardi PP, Riccardi G, Storey RF, Wood D; European Society of Cardiology (ESC); European Atherosclerosis Society (EAS) (2011) ESC/EAS Guidelines for the management of dyslipidaemias The Task Force for the management of dyslipidaemias of the European Society of Cardiology (ESC) and the European Atherosclerosis Society (EAS). Atherosclerosis 217: 3-46.
• Chang PY, Lu SC, Su TC, Chou SF, Huang WH, Morrisett JD, Chen CH, Liau CS, Lee YT (2004) Lipoprotein-X reduces LDL atherogenicity in primary biliary cirrhosis by preventing LDL oxidation. J Lipid Res 45: 2116-2122.
• Crook MA (2013) Lipoprotein X: clinical implications. Ann Clin Biochem 50: 93-94. doi: 10.1177/0004563213478804.
• Edwards CM, Otal MP, Stacpoole PW (1993) Lipoprotein-X fails to inhibit hydroxymethyl glutaryl coenzyme A reductase in HepG2 cells. Metabolism 42: 807-813.
• Glomset JA, Nichols AV, Norum KR, King W, Forte T (1973) Plasma lipoproteins in familial lecithin: cholesterol acyltransferase deficiency. Further studies of very low and low density lipoprotein abnormalities. J Clin Invest 52: 1078-1092.
• Herzum I, Giehl C, Soufi M, Junclas H, Wahl HG (2007) Interference in a homogeneous assay for low-density lipoprotein cholesterol by lipoprotein X. Clin Chem Lab Med 45: 667-671.
• Jahn CE, Schaefer EJ, Taam LA, Hoofnagle JH, Lindgren FT, Albers JJ, Jones EA, Brewer HB Jr. (1985) Lipoprotein abnormalities in primary biliary cirrhosis: association with hepatic lipase inhibition as well as altered cholesterol esterification. Gastroenterology 89: 1266-1278.
• Kaplan M (1996) Primary biliary cirrhosis. N Engl J Med 335: 1570-1580.
• Kuster GM, Drexel H, Bleisch JA, Rentsch K, Pei P, Binswanger U, Amann FW (1994) Relation of cyclosporine blood levels to adverse effects on lipoproteins. Transplantation 57: 1479-83.
• National Cholesterol Education Program (2002) Third report of the National Cholesterol Education Program (NCEP) Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults (Adult Treatment Panel III) final report. Circulation 106: 3143-3421.
• Nikkilä K, Åberg F, Isoniemi H (2014) Transmission of LDLR mutation from donor through liver transplantation resulting in hypercholesterolemia in the recipient. Am J Transplant 14: 2898-2902. doi: 10.1111/ajt.12961.
• Ritland S (1975) The abnormal 'lipoprotein of cholestasis', lipoprotein-X. Scand J Gastroenterol 10: 785-789.
• Rosenson RS, Baker AL, Chow MJ, Hay RV (1990) Hyperviscosity syndrome in a hypercholesterolemic patient with primary biliary cirrhosis. Gastroenterology 98: 1351-1357.
• Sholter DE, Armstrong PW (2000) Adverse effects of corticosteroids on the cardiovascular system. Can J Cardiol 16: 505-511.
• Tashiro T, Mashima Y, Yamamori H, Sanada M, Nishizawa M, Okui K (1992) Intravenous intralipid 10% vs. 20%, hyperlipidemia and increase in lipoprotein X in humans. Nutrition 8: 155-160.
• Toren A, Nagler A (1996) Solitary pulmonary cholesteroloma, multiple xanthelasmas, lipemia retinalis complicating hypercholesterolemia after bone marrow transplantation. Bone Marrow Transplant 18: 457-459.
• Turchin A, Wiebe DA, Seely EW, Graham T, Longo W, Soiffer R (2005) Severe hypercholesterolemia mediated by lipoprotein X in patients with chronic graft-versus-host disease of liver. Bone Marrow Transplant 35: 85-89.
• Witt J, Ober M (1976) Lp-X in newborns: incidence of positive tests without cholestasis. J Clin Chem Clin Biochem 14: 197-202.
• Yeh H, Kitchens WH, Elias N, Kelsey PB, Markmann JF, Hertl M (2011) Hyperlipidemia due to biliary stricture after living-donor liver transplantation. Transplantation 92: e29-e30 doi: 10.1097/TP.0b013e31822d095d.
• Zidan H, Lo S, Wiebe D, Talano J, Alemzadeh R (2008) Severe hypercholesterolemia mediated by lipoprotein X in a pediatric patient with chronic graft-versus-host disease of the liver. Pediatr Blood Cancer 50: 1280-1281. doi: 10.1002/pbc.21522.

Identifiers

DOI

10.18388/abp.2015_971