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视神经脊髓炎谱系疾病的治疗性血浆置换:来自印度北部三级护理中心的经验。

Therapeutic Plasma Exchange in Neuromyelitis Optica Spectrum Disorders-Experience from Tertiary Care Center in North India.

机构信息

Department of Transfusion Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Department of Paediatric Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

出版信息

Neurol India. 2022 Mar-Apr;70(2):710-713. doi: 10.4103/0028-3886.344678.

Abstract

BACKGROUND

Therapeutic plasma exchange (TPE) is indicated in the acute presentation of neuromyelitis optica spectrum disorders (NMOSD) as category II indication as per American Society for Apheresis guidelines 2016.

AIM

To assess the efficacy of TPE in symptomatic patients of NMOSD not responding to high dose intravenous steroids.

PATIENTS AND METHODS

We retrospectively reviewed the record of TPE procedures in NMOSD patients over a period of 3 years (2013-2016). TPEs were done on Cobe® spectra (Terumo BCT, Lakewood Co., USA) using 5% human serum albumin or fresh frozen plasma as replacement fluid. Clinical improvement was recorded post-procedure and adverse reactions if any was noted.

RESULTS

We performed TPE in 11 clinically diagnosed patients of NMOSD. Three (27%) patients had only visual symptoms, five (46%) had both visual as well as muscular symptoms, whereas three (27%) patients presented with only muscular symptoms. Out of seven tested, three patients were aquaporin-4-immunoglobulin-G-positive (AQP4-IgG positive). Visual symptoms improved from no vision pre-TPE therapy to finger counting or 6/24 post-therapy.The muscle power of the limbs improved by grade one to grade two post-therapy. Adverse events were observed in 8% (5/62) of the procedures; allergic reactions to replacement fluid as the most common event (n = 3) followed by hypotension (n = 2). Follow-up was available in 81% (9/11) patients. Two patients died at 3 and 12 months of follow-up. One patient had relapsed and underwent a second TPE cycle.

CONCLUSION

The addition of TPE as a part of therapy is effective and safe in acute exacerbations of NMOSD.

摘要

背景

根据美国体外治疗协会 2016 年的指南,在视神经脊髓炎谱系疾病(NMOSD)的急性发作中,治疗性血浆置换(TPE)被列为 II 类适应证。

目的

评估 TPE 在对大剂量静脉类固醇治疗无反应的 NMOSD 症状性患者中的疗效。

患者和方法

我们回顾性分析了 3 年内(2013-2016 年)NMOSD 患者 TPE 治疗的记录。TPE 使用 Cobe® spectra(美国特鲁姆波生物科技公司)进行,使用 5%人血清白蛋白或新鲜冷冻血浆作为置换液。记录治疗后的临床改善情况,如果有任何不良反应也会记录下来。

结果

我们对 11 例临床诊断为 NMOSD 的患者进行了 TPE。3 例(27%)患者仅有视觉症状,5 例(46%)患者既有视觉症状又有肌肉症状,3 例(27%)患者仅有肌肉症状。在 7 例检测中,3 例患者水通道蛋白 4 免疫球蛋白 G 阳性(AQP4-IgG 阳性)。治疗前,有视觉症状的患者视力为无光感,治疗后视力提高至指数或 6/24。治疗后,四肢肌肉力量提高了 1 级至 2 级。8%(5/62)的操作中观察到不良事件;最常见的事件是对置换液的过敏反应(n=3),其次是低血压(n=2)。81%(9/11)的患者可获得随访。2 例患者在随访 3 个月和 12 个月时死亡。1 例患者复发并接受了第二次 TPE 治疗。

结论

在 NMOSD 的急性加重期,添加 TPE 作为治疗的一部分是有效且安全的。

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