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难治性寻常型天疱疮与高强度体外光化学疗法:一例报告

Refractory pemphigus vulgaris and high-intensity extracorporeal photopheresis: A case report.

作者信息

Castillo-Aleman Yandy Marx, Bencomo-Hernandez Antonio Alfonso, Ventura-Carmenate Yendry, Villegas-Valverde Carlos Agustin, Rivero-Jimenez Rene Antonio

机构信息

Clinical Immunology Specialist.

Health Sciences, Professor in Immuno-Hematology.

出版信息

Photodermatol Photoimmunol Photomed. 2022 Sep 2. doi: 10.1111/phpp.12834.

Abstract

A 41-year-old man with oral pemphigus vulgaris (PV) presented to our clinic with a history of no response to numerous immunosuppressant agents and was referred for extracorporeal photopheresis (ECP) therapy. Although the patient underwent a high-intensity ECP regimen for five months, which included two different photopheresis systems, his oral dysesthesia continued to interfere with oral intake, leading to continued weight loss and other adverse events. The intervention was associated with changes in several immune cell subpopulations without modifying the anti-epidermal antibody titers, aligned with his poor clinical outcome. To the best of the authors' knowledge, this is the first report to examine immunophenotyping of a PV patient who was refractory to previous immunosuppression and recalcitrant to high-intensity ECP therapy.

摘要

一名41岁的寻常型天疱疮(PV)男性患者前来我院就诊,他曾使用多种免疫抑制剂均无反应,遂转诊接受体外光化学疗法(ECP)治疗。尽管该患者接受了为期五个月的高强度ECP方案,其中包括两种不同的光化学疗法系统,但其口腔感觉异常仍持续影响经口摄入,导致体重持续下降及其他不良事件。该干预措施与多个免疫细胞亚群的变化相关,但未改变抗表皮抗体滴度,这与他不佳的临床结局相符。据作者所知,这是首篇对先前免疫抑制难治且对高强度ECP治疗顽固不化的PV患者进行免疫表型分析的报告。

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