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儿童类中毒性表皮坏死松解症/史蒂文斯-约翰逊综合征急性移植物抗宿主病感染及眼部并发症的管理:一项临床病例研究及文献综述

Management of infection and ocular complications in pediatric SJS/TEN-like acute graft-versus-host disease: a clinical case study and literature review.

作者信息

Yan Huimin, Mo Yunjun, Li Yue, Li Qian, Luo Liping, Meng Qing, Jia Lei, Zhou Lintao, Xiao Lixia, Fu Xiaoying

机构信息

Department of Laboratory Medicine, Shenzhen Children's Hospital, Shenzhen, GuangDong, China.

Center of Clinical Laboratory, Shenzhen Hospital, Southern Medical University, Shenzhen, GuangDong, China.

出版信息

Front Immunol. 2025 Jun 16;16:1588297. doi: 10.3389/fimmu.2025.1588297. eCollection 2025.

DOI:10.3389/fimmu.2025.1588297
PMID:40589744
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12206640/
Abstract

Acute graft-versus-host disease (aGVHD) with skin manifestations reminiscent of Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) is associated with poor outcomes. However, optimal management strategies to enhance quality of life in SJS/TEN-like aGVHD remain undefined. This study aims to investigate the management of complex infections and acute ocular injury in patients with SJS/TEN-like aGVHD following allogeneic hematopoietic stem cell transplantation. We conducted a comprehensive analysis of the treatment course for a patient with SJS/TEN-like aGVHD, complemented by a literature review on acute ocular complications and their management in aGVHD patients. A patient diagnosed with grade IV skin aGVHD received effective treatment for multidrug-resistant using minocycline, aztreonam, and ceftazidime-avibactam. Combination therapy with liposomal amphotericin B and voriconazole was efficacious against mixed fungal infections. Immunological assessments indicated reduced lymphocyte counts and increased myeloid-derived suppressor cells, with elevated CD4 PD-1 exhausted and memory cells, reflecting a complex interplay of immune hyperactivity and suppression. A literature review showed that although age, gender, and transplant circumstances were not associated with ocular symptoms, grade II+ cutaneous aGVHD emerged as a key risk factor for conjunctival involvement, characterized by exudation and pseudomembrane formation. Topical glucocorticoids, tacrolimus and cyclosporine eye drops were effective, necessitating regular pseudomembrane removal. Evaluating drug susceptibility and immune status is vital for formulating precise therapies. Early recognition and management of ocular symptoms in SJS/TEN-like aGVHD are essential to prevent irreversible damage.

摘要

伴有类似史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)皮肤表现的急性移植物抗宿主病(aGVHD)与不良预后相关。然而,提高SJS/TEN样aGVHD患者生活质量的最佳管理策略仍不明确。本研究旨在调查异基因造血干细胞移植后SJS/TEN样aGVHD患者复杂感染和急性眼损伤的管理。我们对一名SJS/TEN样aGVHD患者的治疗过程进行了全面分析,并辅以关于aGVHD患者急性眼部并发症及其管理的文献综述。一名被诊断为IV级皮肤aGVHD的患者使用米诺环素、氨曲南和头孢他啶-阿维巴坦接受了针对多重耐药菌的有效治疗。脂质体两性霉素B和伏立康唑联合治疗对混合真菌感染有效。免疫评估显示淋巴细胞计数减少,髓源性抑制细胞增加,CD4 PD-1耗竭和记忆细胞升高,反映了免疫亢进和抑制的复杂相互作用。文献综述表明,虽然年龄、性别和移植情况与眼部症状无关,但II+级皮肤aGVHD是结膜受累的关键危险因素,其特征为渗出和假膜形成。局部糖皮质激素、他克莫司和环孢素滴眼液有效,需要定期清除假膜。评估药物敏感性和免疫状态对于制定精确的治疗方案至关重要。早期识别和管理SJS/TEN样aGVHD中的眼部症状对于预防不可逆损伤至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3349/12206640/66b1ec31872d/fimmu-16-1588297-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3349/12206640/063f8bf65f0b/fimmu-16-1588297-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3349/12206640/11c1fcc15139/fimmu-16-1588297-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3349/12206640/d8c10bcbfaf8/fimmu-16-1588297-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3349/12206640/66b1ec31872d/fimmu-16-1588297-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3349/12206640/063f8bf65f0b/fimmu-16-1588297-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3349/12206640/11c1fcc15139/fimmu-16-1588297-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3349/12206640/d8c10bcbfaf8/fimmu-16-1588297-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3349/12206640/66b1ec31872d/fimmu-16-1588297-g004.jpg

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Case report: Toxic epidermal necrolysis as a unique presentation of acute graft versus host disease in a pediatric patient.病例报告:中毒性表皮坏死松解症作为一名儿科患者急性移植物抗宿主病的独特表现。
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