TREATMENT OF HOMOZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA WITH LOW-DENSITY-LIPOPROTEIN APHERESIS - A 4 YEAR FOLLOW-UP-STUDY

Hypercholesterolemia and elevated lipoprotein (a) (Lp[a]) levels are c onsidered to be risk factors for the development and progression of pr emature atherosclerosis. The purpose of our report is to describe the effects of low density lipoprotein (LDL) apheresis (Liposorber system, Kanegafuchi Chemical Industrial Company LTD, Osaka, Japan) on serum l ipoprotein concentrations and the clinical status in 2 male patients w ith homozygous familial hypercholesterolemia. Compared with pretreatme nt values, the posttreatment concentrations of total cholesterol, LDL cholesterol, and Lp(a) were significantly reduced by 50-60% (p < 0.000 1). The concentration of high density lipoprotein (HDL) cholesterol wa s slightly affected. After one treatment session, LDL cholesterol and Lp(a) were decreased on average by 65% and then increased to reach abo ut 70-75% of the pretreatment values before the next session. Prior to the treatment with LDL apheresis, each patient had suffered one myoca rdial infarction and had had 2 coronary angiographies. After treatment with LDL apheresis, neither cardiac complaints nor myocardial infarct ion were observed. The xanthomas were much decreased during the treatm ent or disappeared. We conclude that LDL apheresis can be continued sa fely and without major technical problems for several years. Apheresis effectively lowers the serum levels of total and LDL cholesterol. Fur thermore, it reduces Lp(a), which is not influenced by lipid-lowering drugs. The reduction of LDL cholesterol and Lp(a) may delay the progre ssion of the atherosclerotic process, thereby helping to reduce the ri sk of new episodes of coronary heart disease and thus extending the li fe expectancy in these patients.