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Lipoprotein Apheresis for a Patient with Sitosterolemia

Description

Sitosterolemia is a rare genetic disorder with excessive absorption of plant sterols. Untreated patients develop tendon xanthomas and premature coronary artery disease. Lipoprotein apheresis can serve as adjunctive therapy to reduce total body stores of the plant sterols.


Management of sitosterolemia may include:

(1) diet low in plant and seafood sterols

(2) lipoprotein apheresis

(3) therapy with ezetimibe to reduce GI absorption of cholesterol and plant sterols

(4) therapy with cholestyramine

 

Lipoprotein apheresis uses a dextran sulfate cellulose column. Apheresis is performed weekly with the volume processed dependent on the clinical response.


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