Aubert I, Bombail D, Erlich D, Goy-Loeper J, Chanu B, Bussel A, Rouffy J
Service de Médecine Interne et Pathologie Vasculaire, Hôpital Saint-Louis, Paris.
Ann Med Interne (Paris). 1988;139 Suppl 1:72-6.
Two patients-a 32 year old man with severe heterozygote familial hyperlipoproteinemia (FH) and a 9 years old girl with homozygote FH-were treated over eight months by LDL apheresis using dextran sulfate cellulose column (Liposorber, Kaneka, Japon). Plasma was separated from blood cells by filtration (TPE Cobe) or centrifugation (2,997 Cobe) through peripheral veins. An IV bolus of 10 IU/kg heparin was given together with local anti-coagulation with 55 g/l sodium citrate, 20 g/l citric acid at a ratio 1:25. Albumin supply was unnecessary. Plasma was removed every 2 weeks through liposorber LA 40 in the adult, and every week through liposorber LA 40 then 2 LA 15 in the child, mean plasma volume exchanged being 1.2 litres in the adult and 1.5 litres par session in the child.
the DSC column removed on the average 60 p. 100 of total cholesterol (TC) and 65 p. 100 of LDL.C. Apoproteins B levels were reduced by 58 p. 100. After each procedure there was a rapid increase in lipid levels to about the 80 to 90 p. 100 of pretreatment value. In the adult, we obtained levels of TC of less than 300 mg/dl with exchanges every 2 weeks combined with an HMG CoA reductase inhibitor (40 mg/day); in the child, with exchanges every week the same inhibitor did not permit a prolongation of the interval between 2 aphereses.
this was confirmed by elution of DSC column bound lipoproteins by 0.1 mol/l NaCl solution. However, the average removal of HDL.C and apoprotein A1 was respectively 31 p. 100 and 32 p. 100. Triglycerides levels were also reduced (48 p. 100).
this was good in both cases. Using the filtration technic, hypotension was reported; this side effect did not appear with centrifugation. In the child, we observed immediate type reactions: nasal obstruction, headache and abdominal pain. The change in plasma protein concentration was caused by dilution and/or non specific absorption.
LDL apheresis alone or combined with an HMG CoA reductase inhibitor is a safe technic, simple to manage without special equipment and producing marked LDL.C level reduction. However, there is also a reduction of HDL-C levels. Despite its high cost, it is a promising new approach to the treatment of FH.
两名患者——一名32岁患有严重杂合子家族性高脂蛋白血症（FH）的男性和一名9岁患有纯合子FH的女孩，使用硫酸葡聚糖纤维素柱（Liposorber，Kaneka，日本）进行了为期八个月的低密度脂蛋白单采治疗。通过外周静脉过滤（TPE Cobe）或离心（2997 Cobe）从血细胞中分离血浆。静脉推注10 IU/kg肝素，并同时用55 g/l柠檬酸钠、20 g/l柠檬酸以1:25的比例进行局部抗凝。无需补充白蛋白。成人每2周通过Liposorber LA 40去除血浆，儿童每周通过Liposorber LA 40然后每两周通过2个LA 15去除血浆，成人平均每次交换血浆量为1.2升，儿童每次为1.5升。
DSC柱平均去除总胆固醇（TC）的60%和低密度脂蛋白胆固醇（LDL-C）的65%。载脂蛋白B水平降低了58%。每次治疗后血脂水平迅速升高至治疗前值的80%至90%。在成人中，每2周进行一次血浆置换并联合使用HMG CoA还原酶抑制剂（40 mg/天），我们将TC水平降至300 mg/dl以下；在儿童中，每周进行血浆置换，同样的抑制剂并不能延长两次单采之间的间隔时间。
通过用0.1 mol/l NaCl溶液洗脱DSC柱结合的脂蛋白证实了这一点。然而，HDL-C和载脂蛋白A1的平均去除率分别为31%和32%。甘油三酯水平也降低了（48%）。
在这两种情况下安全性都良好。使用过滤技术时，有低血压的报告；离心时未出现这种副作用。在儿童中，我们观察到即刻型反应：鼻塞、头痛和腹痛。血浆蛋白浓度的变化是由稀释和/或非特异性吸收引起的。
单独进行低密度脂蛋白单采或与HMG CoA还原酶抑制剂联合使用是一种安全的技术，无需特殊设备即可简单操作，并能显著降低LDL-C水平。然而，HDL-C水平也会降低。尽管成本高昂，但它是治疗FH的一种有前景的新方法。
