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. 2015 Aug 1;10(4):305-312.
doi: 10.2217/clp.15.23.

Management of lipoprotein X and its complications in a patient with primary sclerosing cholangitis

Eric J Brandt  1 , Shane M Regnier  2 , Edward Ky Leung  3 , Sharon H Chou  4 , Beverly W Baron  3 , Helen S Te  5 , Michael H Davidson  6 , Robert M Sargis  7
Affiliations

Management of lipoprotein X and its complications in a patient with primary sclerosing cholangitis

Eric J Brandt et al. Clin Lipidol. .

Abstract

Lipoprotein X (LpX) is an abnormal lipoprotein found in conditions such as lecithin:cholesterol acyltransferase deficiency and cholestatic states (e.g., primary biliary cirrhosis and primary sclerosing cholangitis). Management of severe hypercholesterolemia due to LpX with drugs and physical removal methods is not well established in the literature. A case is discussed of a 51-year-old woman who presented with multiple electrolyte abnormalities, xanthomas and neuropathy found to be secondary to LpX in the setting of primary sclerosing cholangitis. This case highlights that oral medications, including statins, may be insufficient to normalize lipid levels or improve clinical symptoms of LpX and presents therapeutic plasma exchange as a safe and effective therapeutic option to treat the morbid sequela of LpX hyperlipidemia.

Keywords: cholestasis; hypercholesterolemia; lipoprotein X; neuropathy; plasma exchange; primary sclerosing cholangitis; statin; xanthoma.

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Figures

Figure 1
Figure 1. Lipoprotein electrophoresis
Compared with a control sample, the patient’s lipoprotein electrophoresis pattern demonstrates the presence of LpX. LpX: Lipoprotein X.
Figure 2
Figure 2. Planar xanthomas of the palms
(A) Planar xanthomas of the hand prior to therapeutic plasma exchange. (B) Resolution of the planar xanthomas that occurred post-therapeutic plasma exchange and that was accompanied by resolution of her neuropathic pain.
Figure 3
Figure 3. Biochemical response to therapeutic interventions
(A) Demonstrates the patient’s lipid parameters and their response to therapeutic interventions. (B) Reveals changes in hepatic function studies with lipid-directed interventions. LDL-C concentrations were calculated by the Friedewald equation. Because LpX migrates with LDL, the LDL-C concentration reflects both true LDL-C as well as LpX. Solid arrows indicate occurrences of plasma exchange; dashed arrows indicate liver transplantation. HDL-C: HDL-cholesterol; LDL-C: LDL-cholesterol; LpX: Lipoprotein X.
See this image and copyright information in PMC

References

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