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冷球蛋白血症与双重滤过血浆置换:个人经验与文献回顾。

Cryoglobulinemia and double-filtration plasmapheresis: Personal experience and literature review.

机构信息

Department of Nephrology, Hemodialysis, Apheresis, and Kidney Transplantation, Grenoble University Hospital, Grenoble, France.

Grenoble Alpes University, Grenoble, France.

出版信息

Ther Apher Dial. 2023 Feb;27(1):159-169. doi: 10.1111/1744-9987.13885. Epub 2022 May 28.

DOI:10.1111/1744-9987.13885
PMID:35583180
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10084379/
Abstract

BACKGROUND

Cryoglobulinemia is defined as the presence of an abnormal immunoglobulin that may be responsible for vasculitis of small-caliber vessels. Apheresis can be used in order to temporarily eliminate circulating cryoglobulins. The aim of this study was to assess the effectiveness of apheresis (double-filtration plasmapheresis-DFPP-) in symptomatic and/or severe cryoglobulinemias.

METHODS

Four male patients presenting cryoglobulinemic vasculitis and who received DFPP sessions were included.

RESULTS

Their mean age was 57 ± 15 years. One patient had hepatitis-C virus (HCV)-related cryoglobulinemia and the other three patients were carriers of an IgM Kappa monoclonal gammopathy. Mean duration of follow-up was 15 ± 2 months. DFPP allowed healing of ulcerative skin lesions in the first patient and remission of nephrotic syndrome in the other patients after a median of 6(5-10) sessions.

CONCLUSION

DFPP can be used safely in cryoglobulinemic-vasculitis and can be considered early to achieve a faster and sustained clinical-biological response.

摘要

背景

冷球蛋白血症定义为存在异常免疫球蛋白,可能导致小血管血管炎。可以采用血浆置换(双重滤过血浆置换-DFPP-)来暂时清除循环冷球蛋白。本研究旨在评估血浆置换(双重滤过血浆置换-DFPP-)在有症状和/或严重冷球蛋白血症中的疗效。

方法

纳入了 4 名患有冷球蛋白血症血管炎并接受 DFPP 治疗的男性患者。

结果

他们的平均年龄为 57±15 岁。1 名患者为丙型肝炎病毒(HCV)相关冷球蛋白血症,另外 3 名患者为 IgM Kappa 单克隆丙种球蛋白血症携带者。中位随访时间为 15±2 个月。DFPP 使第 1 名患者的溃疡性皮肤病变愈合,其他 3 名患者的肾病综合征在 6(5-10)次治疗后缓解。

结论

DFPP 可安全用于冷球蛋白血症血管炎,可早期应用以获得更快和持续的临床-生物学反应。

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