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Lp8 is potentially associated with partial lecithin:cholesterol acyltransferase deficiency in a patient with primary
Toshiyuki Watanabe1, Toshirou Nema1, Nobuyuki Hiruta2
1Department of Clinical-Laboratory and Experimental-Research Medicine, Toho University Sakura Medical Center, Sakura, Japan; LSI Medience Corporation, Tokyo, Japan.
Background:
The significance of Lp8, that is, abnormal lipoprotein(s) detected in fraction 8 by combined high-performance liquid chromatography/gel filtration column in patients with familial lecithin:cholesterol acyltransferase (LCAT) syndrome, in relation to the severity of LCAT deficiency has not been analyzed.
Objective:
We have studied Lp8 in a patient with primary biliary cirrhosis.
Methods:
Plasma lipoproteins were analyzed using high-performance liquid chromatography/gel filtration column in the course of treatment of a 47-year-old female patient with primary biliary cirrhosis.
Results:
Electrophoretic lipoprotein analyses showed massive accumulation of abnormal ß- and preß-lipoproteins with a minor lipoprotein fraction at a position near the cathode corresponding to Lp-X, on day A (status: hypercholesterolemia, LCAT activity undetectable). Chromatographic lipoprotein subfraction analysis revealed free cholesterol- and phospholipid-rich lipoproteins in fractions 1-6, corresponding to chylomicrons and very low-density lipoprotein, and phospholipid- and triglyceride-rich lipoproteins with increased free cholesterol, that is, Lp8, in fractions 7-9 (corresponding to low-density lipoprotein). On day B, after additional treatment for 7 months (status: almost normolipidemia, decreased LCAT activity), although the abnormal lipoprotein and the lipoproteins in fractions 1-6, were drastically decreased, the presence of Lp8 persisted.
Conclusions:
Lp8 likely is a minor abnormal lipoprotein fraction in patients with mildly decreased secondary LCAT activity, as well as with severely reduced primary LCAT activity.
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