Regression of Atherosclerosis in Homozygous Familial Hypercholesterolemia by Plasma Exchange Therapy
Year: 1987
Type: article
Abstract: Three patients in members of the same family (patient 1, 38yrs. male; patient 2, 41yrs. female; patient 3, 44yrs. female) with homozygous familial hypercholesterolemia were treated by repeated plasma exchange for several years. The effectiveness of long-term plasmapheresis for homozygous familial hypercholesterolemia has been evaluated in three patients receiving hypolipidemic dugs.Their serum cholesterol levels were 596±40.0 (3), 719±39.5 (3) and 661±25.7 (3)mg/dl (MSD), respectively. After 5 years of probucol and plasma exchange at 2 weekly intervals, the mean serum cholesterol in patient 1 before exchange was 427±24mg/dl and the mean value after exchange was 137±18mg/dl. In patient 2 after 6 years of plasma exchange and probucol, the mean serum cholesterol before exchange was 533±21mg/dl and after exchange was 143±16mg/dl. In patient 3 after 6 years of plasma exchange and probucol, pre-exchange value was 409±15mg/dl and postexchange value was 162±11mg/dl. In patient 2, aortocoronary angiographic appearances were unchanged during 3 years of plasmapheresis therapy. In patient 3, the irregular appearance of the aorta was improved and the irregular appearance of the right coronary artery was unchanged during 3 years of plasma exchange. In patient 1, an initial coronary angiogram showed a 50% stenosis in the left anterior descending (LAD, seg. 8) and the left circumflex (CX, seg. 13) artery. After one year of 2L plasma exchange and cholestyramine, stenosis was much more severe. However, after 4 years of plasmapheresis using double filtration method and probucol, there was a marked improvement in the stenosis of the left anterior descending and the left circumflex artery.We conclude that plasmapheresis and probucol retards the rate of progression of atherosclerotic changes and induces regression of coronary atherosclerosis in homozygotes.
Institution: Sumitomo Hospital
Language: English
Subfield: Cardiology and Cardiovascular Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W577202108