Effective reduction of plasma LDL levels by LDL apheresis in familial defective apolipoprotein B-100.
The clinical response to long-term reduction of the plasma LDL cholesterol concentration was studied in a man with severe coronary artery disease associated with familial defective apolipoprotein B-100 (FDB). Plasma exchange repeated at 2-week intervals, combined with lipid-lowering drugs, led to remission of angina and improved exercise test performance. A similar clinical response was achieved after LDL apheresis with dextran sulphate columns repeated once every 2 weeks in combination with drug treatment. The reduction in plasma LDL cholesterol level brought about by LDL apheresis was at least as marked in the FDB patient as in 5 patients with familial hypercholesterolaemia. We conclude that FDB patients with coronary artery disease may derive clinical benefit from prolonged reduction of their plasma cholesterol levels and that LDL containing apo B-100 in which arginine at position 3500 is replaced by glutamine is removed from plasma by dextran sulphate columns as efficiently as is normal LDL.
Maher
V M
VM
MRC Lipoprotein Team, Hammersmith Hospital, London, UK.
Kitano
Y
Y
Neuwirth
C
C
Gallagher
J J
JJ
Thompson
G R
GR
Myant
N B
NB
D053299:Apolipoprotein B-100; D001055:Apolipoproteins B / Q000737:chemistry*; D001781:Blood Component Removal; D008078:Cholesterol, LDL / Q000097:blood*; D003327:Coronary Disease / Q000097:blood / Q000628:therapy; D005260:Female; D006402:Hematologic Diseases / Q000097:blood / Q000628:therapy*; D006579:Heterozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000235:genetics / Q000628:therapy; D008297:Male; D008875:Middle Aged
D053299:Apolipoprotein B-100; D001055:Apolipoproteins B; D008078:Cholesterol, LDL