RT期刊文章SR电子T1 Anti-MAG相关神经病变患者的特征响应利妥昔单抗(5242)摩根富林明神经病学神经学乔FD Lippincott Williams &威尔金斯SP 5242签证官94 15 A1大卫补充Chaar A1 Mihir Kakara A1 Sh首页itiz Sriwastava A1 Sasan Moshirzadeh A1米甲卤代烷A1罗伯特Lisak年2020 UL //www.ez-admanager.com/content/94/15_Supplement/5242.abstract AB目的:评估患者的长期响应利妥昔单抗和描述的特点,有良好的结果。背景:Anti-myelin相关糖蛋白(MAG)神经病变是一种使人衰弱的paraproteinemic脱髓鞘病变。免疫疗法的效果一直不一致的多个研究;然而,rituximab-a b细胞消耗经纪人被证明是有益的在病人的一个子集。设计/方法:7 anti-MAG神经病变患者利妥昔单抗的病例分析。记录了人口、血清学和电诊法的特性。结果来评估疗效包括改良Rankin规模(夫人)和炎性病变病因和治疗(INCAT)规模。结果:86%的患者都是白人男性。在诊断上,平均年龄57岁,平均anti-MAG效价在急救员和1:513,600 1:40,000无(p = 0.04)。疾病治疗开始前4±3年的反应,相比11±7年无(p = 0.05)。滴度下降了7.5±4.7折后治疗。在12个月的治疗后,病人4/7报道症状改善感觉异常、异常性疼痛、震颤和步态。远端神经系统检查显示复苏强度、振动和本体感受解决伯格的迹象,和下肢反射的复兴。 INCAT (mRS) score improved by 1.8±1.0, (p=0.01) (1.3±0.5, p=0.004) points from a baseline of 3.0±1.6 (2.3±0.5). Terminal latency index (TLI) of the median nerve was 0.30±0.05. The remaining patients did not have further deterioration in their symptoms, exam or scores. One patient had lambda light-chains, unlike the others with kappa light-chains, and did not improve. Another elderly patient with TLI of 0.18 did not respond either despite a 9-fold reduction in anti-MAG titer.Conclusions: Features of rituximab responders include shorter disease-duration, lower anti-MAG titers and TLI>0.25. Non-responders did not deteriorate further and remained stable. This retrospective case-series supports rituximab as a viable option in a subset of patients for management of anti-MAG neuropathy, with improvements in symptoms, neurological exam and functional neuropathy scales.Disclosure: Dr. Chaar has nothing to disclose. Dr. Kakara has nothing to disclose. Dr. Sriwastava has nothing to disclose. Dr. Moshirzadeh has nothing to disclose. Dr. Halon has nothing to disclose. Dr. Lisak has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Alexion, Argenix, Novartis, Syntimmune, Teva Pharmaceuticals (non-branded talks), Alpha Sites, Clearview Consulting, Destum, GLG consulting, Haven Consulting, Informa Consulting, Insights Pharma Consulting, Slingshot Consulting. Dr. Lisak has received research support from Catalyst, Chungai, Genentec/Roche, Mallinckdrot, Medimmune, Novartis, Ra Pharmaceuticals, Teva Phamaceuticals.