Long-term prevention of premature coronary atherosclerosis in homozygous familial hypercholesterolemia.
Homozygous familial hypercholesterolemia (FHH) leads to severe premature atherosclerosis. A 22-year-old woman with FHH has been treated with a combination of H.E.L.P. apheresis (heparin-mediated extracorporeal LDL precipitation) and statins for 15 years. The combined treatment maintained a plasma LDL-cholesterol reduction from baseline of 840 to 122 mg/dL (85% reduction). In addition, H.E.L.P. apheresis reduced the elevated lipoprotein(a) and fibrinogen levels by 60% to 70%. All xanthomata disappeared. There is no evidence of premature atherosclerosis studied by means of electron beam computed tomography and (13)N-ammonia positron emission tomography: The entire coronary vasculature is free of calcifications. Her myocardial blood flow at rest (87 mL/100 g/min) and during stress (308 mL/100 g/min) and the coronary flow reserve (3.5) are normal after H.E.L.P. treatment. This case demonstrates the efficacy and safety of the combined treatment of H.E.L.P. apheresis and statins even in serious cases of FHH.
Jaeger
Beate Roxane
BR
Institute of Clinical Chemistry, Universitätsklinikum Grosshadern, Ludwig-Maximilians-Universität München, Germany.
Tsobanelis
Theoharis
T
Bengel
Frank
F
Schwaiger
Markus
M
Seidel
Dietrich
D
D000328:Adult; D000924:Anticholesteremic Agents / Q000627:therapeutic use*; D001781:Blood Component Removal; D011232:Chemical Precipitation; D008078:Cholesterol, LDL / Q000097:blood; D003131:Combined Modality Therapy; D003324:Coronary Artery Disease / Q000517:prevention & control*; D005112:Extracorporeal Circulation; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D006938:Hyperlipoproteinemia Type II; D008148:Lovastatin / Q000627:therapeutic use*; D016896:Treatment Outcome
D000924:Anticholesteremic Agents; D008078:Cholesterol, LDL; D005343:Fibrinolytic Agents; D006493:Heparin; D008148:Lovastatin