血浆置换治疗视神经脊髓炎谱系疾病及其有效性分析——附4例报告  

Therapeutic safety and efficacy of plasma exchange in patients with neuromyelitis optica spectrum disorder:four cases

作　　者：梁宪红[1] 黄晓霞 曾东杰 张亚南[1] LIANG Xianhong;HUANG Xiaoxia;ZENG Dongjie;ZHANG Yanan(Department of Neurology,China National Clinical Research Center for Neurological Diseases;Department of Blood Transfusion,Beijing Tian Tan Hospital,Capital Medical University,Beijing 100070,China)

机构地区：[1]首都医科大学附属北京天坛医院神经内科国家神经系统疾病临床研究中心,北京100070 [2]首都医科大学附属北京天坛医院输血科,北京100070

出　　处：《中国输血杂志》2020年第1期22-25,共4页Chinese Journal of Blood Transfusion

基　　金：北京市卫生系统学科骨干项目[2014-3-037]

摘　　要：目的探讨血浆置换(PE)对激素治疗无效的视神经脊髓炎谱系疾病(NMOSD)的治疗效果。方法收集本院2016-2017年神经病学中心神经感染与免疫疾病科收治住院的84名NMOSD患者的病历资料,从中选择出因激素治疗无效而采用PE治疗的急性期NMOSD病例,采用回顾性分析方法比较治疗前后扩展残疾状态量表评分(EDSS)的差异,评价PE疗效和副作用,并与仅用大剂量甲泼尼龙冲击治疗NMOSD病例的显效时间、EDSS评分作对比。结果本组中因激素治疗无效而采用PE治疗的急性期NMOSD患者的比例为4.76%(4/84);PE置换量183-1625.4(中位数1118.7)mL/次;经PE治疗后EDSS评分由6.13±0.41降为4.88±0.96(P<0.05),EDSS平均下降1.25分;而经激素冲击治疗后EDSS平均下降0.75分。患者视力、肌力较前好转时间:采用PE者1-3(中位数2.5)d(1例于PE 1.5 h时出现过敏反应,PE治疗后EDSS下降2分);仅采用激素冲击治疗者2-14(中位数6.4)d。本组经PE治疗的NMOSD患者除抗-AQP4,其他免疫抗体,包括抗核抗体、抗-SSA、抗-SSB、RO-52抗体阳性效价明显下降。结论 PE对常规疗法无效的NMOSD具有较快缓解和稳定病情的作用,可能对难治性NMOSD是1种有效、安全的疗法。Objective To investigate the efficacy of plasma exchange(PE) therapy in neuromyelitis optica spectrum disorder(NMOSD) patients suffered from ineffective high-dose steroid pulse therapy. Methods Eighty four cases of NMOSD from 2016 to 2017 in Department of Neurology, Neuroinfection and Neuroimmunology Center in Beijing Tian Tan Hospital were retrospectively studied. Of them, four cases of acute phase NMOSD treated with PE due to ineffective high-dose steroid pulse therapy were selected. The differences in expanded disability status scale(EDSS) before and after PE treatment were compared to evaluate the efficacy and side effects, compared with the effective time and EDSS scores of NMOSD treated with high-dose steroid pulse therapy alone. Results The proportion of patients with acute phase NMOSD treated with PE due to invalid steroid therapy was 4.76%(4/84);PE extracted amount at 183-1 625.4(median 1 118.7) mL/time;After PE treatment, the EDSS decreased from 6.13±0.41 to 4.88±0.96, and the EDSS score decreased by 1.25 score in average. The difference of EDSS between patients before and after treatment was statistically significant. While, the EDSS score decreased by 0.75 score in average after steroid therapy. The patient′s visual acuity and muscle strength were better: PE 1-3(median 2.5) d(1 case had an anaphylaxis at 1.5 h PE, and the EDSS decreased 2 scores after PE treatment);The steroid therapy alone was 2-14(median 6.4) d. Excluding anti-AQP4 antibody in NMOSD patients, other positive immune antibodies were anti-nuclear antibody, anti-SSA antibody, anti-SSB antibody, RO-52, and the antibody titers of positive patients decreased significantly after PE treatment. Conclusion PE therapy in NMOSD patient due to ineffectiveness of conventional therapy, has a faster and more mitigation effect. It may be an effective and safe treatment for refractory NMOSD.

关 键 词：血浆置换 视神经脊髓炎谱系疾病 激素冲击治疗 复发-缓解 治疗 

分 类 号：R457.14[医药卫生—治疗学] R744.5[医药卫生—临床医学]