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Impaired efficacy of selective LDL-apheresis in primary biliary cirrhosis
G Busnach1, G Franceschini, G Chiesa
1Department of Nephrology, Niguarda Ca Granda Hospital, Milano, Italy.
Insights
Low-density lipoprotein apheresis (LDL-apheresis) effectively managed severe dyslipidemia in a primary biliary cirrhosis patient. Cascade filtration demonstrated superior efficacy in removing cholesterol and lipoprotein-X compared to dextran sulfate cellulose adsorption.
Area of Science:
- Cardiology
- Hepatology
- Biochemistry
Background:
- Severe dyslipidemia and lipoprotein-X (LP-X) accumulation can occur in primary biliary cirrhosis (PBC) patients with cholestasis.
- Extracorporeal LDL-apheresis is a potential treatment modality for managing such lipid abnormalities.
Observation:
- A patient with PBC and severe dyslipidemia underwent LDL-apheresis using cascade filtration (DF) or dextran sulfate cellulose adsorption (DSC).
- Initial weekly treatments significantly reduced total cholesterol from 1038 to 430 mg/dl.
- Subsequent bi-weekly treatments maintained cholesterol levels between 438-505 mg/dl until liver transplantation.
Findings:
- Cascade filtration (DF) removed an average of 47.1% of total cholesterol per procedure, compared to 30.0% with DSC.
- DF removed both LDL and LP-X, while DSC primarily lowered LDL.
- Despite differences in plasma volume treated (38.0 ml/kg for DF vs. 49.9 ml/kg for DSC), cholesterol removal kinetics were similar, but DF showed higher efficacy.
Implications:
- Cascade filtration is a highly effective treatment for severe dyslipidemia and LP-X accumulation in cholestatic liver disease.
- DF's ability to remove LP-X makes it a preferred option over DSC for patients with cholestasis-induced lipid disorders.
- This study supports DF as a potential first-line therapy for managing complex lipid profiles in specific liver disease contexts.
Abstract:
Low-density lipoprotein apheresis (LDL-apheresis) was done with either cascade filtration (DF) or dextran sulfate cellulose adsorption (DSC) in a patient with primary biliary cirrhosis who developed severe dyslipidemia associated with cholestasis and accumulation of lipoprotein-X (LP-X). The extracorporeal treatment was initially performed weekly, and resulted in a sharp drop in total cholesterol from 1038 to 430 mg/dl. During the next four months the patient was treated every 10-15 days, and pre-apheresis cholesterol levels were maintained between 438 and 505 mg/dl, until an orthotopic liver transplantation was successfully performed. With semi-selective DF a mean 47.1% of total cholesterol was removed per procedure compared to 30.0% with DSC, although the volume of treated plasma was 38.0 vs 49.9 ml/kg body weight. The changes in plasma cholesterol levels during DSC and DF showed that the kinetics of cholesterol removal were similar with both techniques, but the efficacy differed; DF removed both LDL and LP-X from plasma, whereas DSC selectively lowered the LDL content. Cascade filtration may therefore be considered as a first-choice treatment for patients with LP-X accumulation due to cholestasis.