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神经系统疾病患者的治疗性血浆置换:63例病例回顾

Therapeutic Plasma Exchange in Patients with Neurologic Disorders: Review of 63 Cases.

作者信息

Tombak Anil, Uçar Mehmet Ali, Akdeniz Aydan, Yilmaz Arda, Kaleagası Hakan, Sungur Mehmet Ali, Tiftik Eyup Naci

机构信息

Department of Hematology, Mersin University Faculty of Medicine, Çiftlikköy Kampüsü, Mersin, Turkey.

Department of Neurology, Mersin University Faculty of Medicine, Mersin, Turkey.

出版信息

Indian J Hematol Blood Transfus. 2017 Mar;33(1):97-105. doi: 10.1007/s12288-016-0661-3. Epub 2016 Feb 22.

Abstract

Therapeutic plasma exchange (TPE) is a procedure that reduces circulating autoantibodies of the patients. TPE is commonly used in neurological disorders where autoimmunity plays a major role. We report our experience with regard to the indications, adverse events and outcomes of plasma exchange in neurological disorders. Sixty-three patients were included to this retrospective study. Median age was 48 years (range 1-85), there was a predominance of males. Neurological indications included Guillain-Barrè syndrome ( = 22), myasthenia gravis ( = 21), chronic inflammatory demyelinating polyneuropathy ( = 7), polymyositis ( = 3), multifocal motor neuropathy ( = 2), acute disseminated encephalomyelitis ( = 2), neuromyelitis optica ( = 2), multiple sclerosis ( = 2), limbic encephalitis ( = 1) and transverse myelitis ( = 1). TPE was frontline therapy in 57 % of the patients ( = 36). Total number of TPE sessions was 517; median number of sessions per patient was 8 (range 1-66). TPE was done through a central venous access in 97 % and through a peripheral venous access in 3 % of the patients. Human albumin was used as replacement fluid in 49 %, hydroxyethyl starch (HES) in 49 % and fresh frozen plasma in 2 % of the cases. Adverse reactions were recorded in 60 % of the patients. Total ratio of complications in 517 TPE procedures was 10.8 % and these were mild and manageable such as allergic reactions and hypotension. Overall response rate was 81 %. Interestingly, complication and response rates were similar in both HES and human albumin groups. We conclude that TPE is an effective treatment in neurologic diseases in which autoimmunity plays an important role in the pathogenesis and HES can be used instead of albumin as replacement fluid in these disorders, since it is cost-effective, has similar efficacy and complication rates.

摘要

治疗性血浆置换(TPE)是一种可减少患者循环自身抗体的治疗方法。TPE常用于自身免疫起主要作用的神经系统疾病。我们报告了我们在神经系统疾病中进行血浆置换的适应证、不良事件及治疗结果方面的经验。本回顾性研究纳入了63例患者。中位年龄为48岁(范围1 - 85岁),男性居多。神经科适应证包括吉兰 - 巴雷综合征(n = 22)、重症肌无力(n = 21)、慢性炎症性脱髓鞘性多发性神经病(n = 7)、多发性肌炎(n = 3)、多灶性运动神经病(n = 2)、急性播散性脑脊髓炎(n = 2)、视神经脊髓炎(n = 2)、多发性硬化(n = 2)、边缘性脑炎(n = 1)和横贯性脊髓炎(n = 1)。57%(n = 36)的患者将TPE作为一线治疗。TPE总疗程数为517次;每位患者的中位疗程数为8次(范围1 - 66次)。97%的患者通过中心静脉通路进行TPE,3%的患者通过外周静脉通路进行。49%的病例使用人白蛋白作为置换液,49%使用羟乙基淀粉(HES),2%使用新鲜冰冻血浆。60%的患者记录到不良反应。517次TPE操作的总并发症发生率为10.8%,且这些并发症较轻且易于处理,如过敏反应和低血压。总体有效率为81%。有趣的是,HES组和人白蛋白组的并发症发生率和有效率相似。我们得出结论,TPE是自身免疫在发病机制中起重要作用的神经系统疾病的有效治疗方法,并且在这些疾病中HES可替代白蛋白作为置换液,因为它具有成本效益,疗效和并发症发生率相似。

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