Plasma exchange and low-density lipoprotein filtration therapy in heterozygous familial hypercholesterolemia

G.J. Tijhuis, J.J. van Doormaal MD, L. Rijskamp, C.Th.Smit Sibinga MD, PhD, H. Doorenbos MD, PhD
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引用次数: 1

Abstract

We studied serum lipids during and shortly after a single plasma exchange (PE) on seven separate occasions in four patients, and during long-term treatment by regular PE in three patients with heterozygous familial hypercholesterolemia. Also, a comparison was made for efficacy in lipid and apolipoprotein removal between long-term weekly PE therapy and three consecutive weekly low-density lipoprotein (LDL) filtration procedures in one patient. PE was done by intermittentflow centrifugation (IFC) with Haemonetics V50 apparatus, exchanging one plasma volume. LDL filtration consisted of a cascade PE, in which the plasma was separated by IFC and the LDL fraction was subsequently removed by membrane filtration over a polyalcohol filter (Evaflux®). From the results, we concluded that (1) LDL cholesterol is reduced about 50% by both methods; (2) after PE, high-density lipoprotein (HDL) cholesterol does not increase during the first two hours, but rises thereafter faster than LDL cholesterol and can reach baseline within three days; (3) by LDL filtration, HDL cholesterol decreases about 30% less than LDL cholesterol and tends to increase above the starting levels found during weekly PE treatment; (4) the reductions in LDL and HDL cholesterol are larger than those in apolipoprotein B and A-I, respectively, in both methods; and (5) connection of the LDL filter device to the Haemonetics V50 system simplifies the cascade procedure, resulting in a single needle approach and avoidance of hemolysis in the primary plasma separation.

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血浆交换和低密度脂蛋白过滤治疗杂合子家族性高胆固醇血症
我们研究了4例患者在7个不同的场合进行单次血浆交换(PE)期间和之后不久的血脂,以及3例杂合子家族性高胆固醇血症患者进行常规PE长期治疗期间的血脂。此外,还比较了一名患者长期每周PE治疗和连续三周低密度脂蛋白(LDL)过滤治疗在脂质和载脂蛋白去除方面的疗效。PE采用Haemonetics V50间歇离心(IFC),交换1个血浆体积。LDL过滤由级联PE组成,其中血浆由IFC分离,LDL部分随后通过多醇过滤器(Evaflux®)的膜过滤去除。结果表明:(1)两种方法均可使低密度脂蛋白胆固醇降低约50%;(2) PE后,高密度脂蛋白(HDL)胆固醇在前2小时内不升高,但此后上升速度快于LDL胆固醇,可在3天内达到基线;(3)通过LDL过滤,HDL胆固醇比LDL胆固醇降低约30%,并有高于每周PE治疗起始水平的趋势;(4)两种方法对低密度脂蛋白和高密度脂蛋白的降低幅度分别大于载脂蛋白B和载脂蛋白A-I的降低幅度;(5)将低密度脂蛋白过滤装置连接到Haemonetics V50系统,简化了级联过程,实现了单针入路,避免了初级血浆分离过程中的溶血。
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