Objective: We evaluated the safety and efficacy of lipoprotein apheresis (LA) in patients with familial hypercholesterolemia (FH) who can't reach low-density lipoprotein cholesterol(LDL-C) target goals with the maximal tolerated dose of lipid-lowering agents. Methods: This was a retrospective cross-sectional study. Between February 2015 and November 2019, patients with FH who were admitted in Fuwai hospital and treated with LA were consecutively enrolled. Based on intensive lipid-lowering agents, these patients received LA by double filtration plasma pheresis (DFPP) method. The changes of lipid levels such as LDL-C and lipoprotein(a)[Lp(a)] were compared before and after LA treatment, and the changes of immunoglobulin (Ig) concentration and LA-related adverse effects were also discussed. Results: A total of 115 patients with FH were enrolled in this study, of which 8 cases were homozygous FH and 107 cases were heterozygous FH. The age was (43.9±12.2) years and there were 75 (65.2%) males, and 108 (93.8%) with coronary artery disease. For pre-and immediately after LA treatment, the LDL-C was (5.20±2.94) mmol/L vs. (1.83±1.08) mmol/L, Lp(a) concentration was 428.70(177.00, 829.50)mg/L vs. 148.90(75.90, 317.00) mg/L (P<0.001), with a decrease of 64.2% and 59.8% respectively. The levels of IgG and IgA measured 1 day after LA treatment were both in the normal range and IgM concentration was below the reference value, the reductions of which were 15.1%, 25.0% and 58.7% respectively (P<0.001). Six patients had mild symptoms of nausea, hypotension dyspnea and palpitation, the symptoms were relieved by symptomatic treatment. Conclusion: For patients with FH who do not achieve LDL-C target goal with the maximal tolerated lipid-lowering agents, especially those with elevated Lp(a) levels, LA, which can significantly further reduce LDL-C and Lp(a) levels, is an effective and safe option.
目的： 探讨对于接受强化降脂药物治疗后低密度脂蛋白胆固醇（LDL-C）不能达标的家族性高胆固醇血症（FH）患者，采用脂蛋白分离即血脂净化（LA）治疗的有效性与安全性。 方法： 本研究为回顾性、横断面研究。连续选取2015年2月至2019年11月于中国医学科学院阜外医院进行LA治疗的FH患者，所有患者均在强化降脂药物治疗的基础上，采用双重滤过血浆置换（DFPP）法进行LA治疗。收集FH患者的年龄、性别、FH类型等基本临床信息，分析LA治疗前后LDL-C、脂蛋白a［Lp（a）］水平变化及其降幅，同时分析免疫球蛋白（Ig）变化以及LA治疗相关不良反应情况。 结果： 共计纳入115例FH患者，其中杂合型FH患者107例（93.0%）、纯合型8例（7.0%）；年龄（43.9±12.2）岁，男性75例（65.2%），合并冠心病108例（93.8%）。LA治疗前LDL-C和Lp（a）水平分别为（5.20±2.94）mmol/L和［428.70（177.00，829.50）］mg/L，LA治疗后即刻LDL-C和Lp（a）水平均显著降低，分别降至（1.83±1.08）mmol/L和148.90（75.90，317.00）mg/L（P均<0.001），降幅分别达64.2%和59.8%。LA治疗后1 d IgG、IgA均可维持于正常水平（降幅分别为15.1%、25.0%，P均<0.001），IgM低于正常水平（降幅58.7%，P<0.001）。6例（5.2%）患者出现LA治疗相关不良反应，包括恶心、低血压、气短、心悸等，程度均轻微，经对症处理后症状缓解。 结论： 对于接受强化降脂药物治疗后LDL-C不达标的FH患者，尤其是伴Lp（a）升高者，DFPP法LA治疗可进一步大幅度降低LDL-C和Lp（a），是一种高效且安全的降脂治疗措施。.