Management of lipoprotein-X accumulation in severe cholestasis by semi-selective LDL-apheresis.
Liver disorders characterized by prolonged bile stasis are often associated with the accumulation of an abnormal lipoprotein, lipoprotein-X (LP-X), in plasma. LP-X is separated in the low-density lipoprotein (LDL) density range, but lacks apolipoprotein B and does not interact with the LDL receptor; LP-X can cause hyperlipidemia, cutaneous xanthomas, and worsening of arterial disease. We report the case of a patient with severe cholestasis, markedly elevated plasma cholesterol levels (26.8 to 31.5 mmol/L), mainly due to a massive accumulation of LP-X in plasma, and diffuse xanthomas. To reduce the elevated cholesterol levels, the patient was given extracorporeal treatment aimed at removing atherogenic lipoprotein (LDL-apheresis). LDL-apheresis was performed at weekly or bi-weekly intervals, either by a semi-selective technique using filters with a defined pore diameter (double filtration, DF) or by a more selective technique using dextran-sulfate-cellulose (DSC) columns able to bind LDL. The semi-selective DF technique proved more effective than DSC, removing 48% of total cholesterol (compared to 30% with DSC), and lowering cholesterol levels to 11.1 mmol/L in 6 weeks. DF removed both LDL and LP-X from plasma, whereas DSC selectively decreased the LDL content. The reduction of plasma cholesterol levels was associated with a complete regression of the xanthomas, supporting DF apheresis as a first-choice treatment for patients with massive LP-X accumulation due to cholestasis.
Franceschini
G
G
Center E. Grossi Paoletti, University of Milano, Italy.
Busnach
G
G
Chiesa
G
G
Sirtori
C R
CR
D000328:Adult; D001781:Blood Component Removal / Q000295:instrumentation / Q000379:methods / Q000592:standards*; D002780:Cholestasis, Intrahepatic / Q000150:complications*; D002784:Cholesterol / Q000097:blood; D005069:Evaluation Studies as Topic; D006801:Humans; D006951:Hyperlipoproteinemias / Q000097:blood / Q000209:etiology / Q000628:therapy*; D008073:Lipoprotein-X / Q000097:blood*; D008074:Lipoproteins / Q000097:blood; D008297:Male; D014280:Triglycerides / Q000097:blood
D008073:Lipoprotein-X; D008074:Lipoproteins; D014280:Triglycerides; D002784:Cholesterol