FHにおけるLDLアフェレシス : 最近の知見(<特集>動脈硬化とアフェレシス)

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Familial hypercholesterolemia (FH) is an autosomal dominant disorder caused by mutation in the gene related to the LDL receptor pathway. Homozygous patients exhibit severe hypercholesterolemia and premature atherosclerosis beginning in their childhood. Atherosclerotic changes in homozygous patients often show a severe form, making these patients' prognosis poor. LDL-apheresis has been developed to prevent atherosclerosis by removal of atherogenic lipoproteins. In Japan, LDL-apheresis treatment is applied in all homozygous patients, and heterozygous patients who exhibit above 250 mg/dl of total cholesterol level with medication and severe coronary artery disease. Here we show our experiences of treating homozygous and heterozygous FH patients using LDL-apheresis. Among eight homozygous patients, two patients have been free from cardiovascular disease and one patient has regressed once. These three patients started LDL-apheresis in childhood. On the other hand, two patients who started LDL-apheresis as adults died of acute myocardial infarction. Among 14 heterozygous patients who withdrew from apheresis therapy, three patients died of cardiovascular disease, whereas, among 12 heterozygous patients who continued apheresis therapy, no patient died of cardiovascular disease. These results suggest that early introduction of LDL-apheresis can prevent atherosclerotic diseases in patients, and that heterozygous patients with severe atherosclerosis should continue LDL-apheresis therapy.
日本アフェレシス学会の論文
2013-02-28

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