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用于木质化结膜炎的冷冻去除血浆输注

Cryo-Depleted Plasma Infusions for Ligneous Conjunctivitis.

作者信息

Bonde Scheel-Bech Anna, Roed Rasmussen Marie Louise, Heegaard Steffen, Schmidt Ida Maria, Stensballe Jakob

机构信息

Department of Ophthalmology, University Hospital, Rigshospitalet, Copenhagen, Denmark.

Eye Pathology Section, Department of Pathology, University of Copenhagen, Copenhagen, Denmark.

出版信息

Case Rep Ophthalmol. 2025 Jul 7;16(1):542-550. doi: 10.1159/000546953. eCollection 2025 Jan-Dec.

Abstract

INTRODUCTION

This case report presents the clinical, histological, and genetic characteristics of a patient with ligneous conjunctivitis and to provide a long-term treatment schedule, using cryo-depleted plasma infusions in combination with topical cyclosporine as a successful therapeutic option.

CASE PRESENTATION

A 12-year-old Turkish girl experienced years of ocular itching, irritation, and increased light sensitivity. Initial clinical examination revealed the presence of pseudomembranes and giant papillae on the tarsal conjunctivae of both eyes. The biopsy of a membrane from the left eye was taken for histological examination. Manifestations of severe comorbidity since infancy (congenital hydrocephalus, asthma, recurrent respiratory infections, and impaired wound healing) and parental consanguinity prompted a genetic evaluation. The pseudomembranes were composed of fibrin, granulation tissue, and inflammatory cells consistent with the diagnosis of ligneous conjunctivitis. The genetic analysis revealed homozygosity for a pathogenic missense variant in plasminogen (PLG), causing PLG deficiency. Due to severe systemic complications, the patient was successfully treated with cryo-depleted plasma infusions and cyclosporine eye drops, resulting in the complete remission of the ligneous conjunctivitis.

CONCLUSION

This case report highlights the importance of considering PLG deficiency in patients with recurring chronic conjunctivitis. Long-term treatment with cryo-depleted plasma infusions in combination with short-term use of topical cyclosporine may provide an efficient therapeutic option for ligneous conjunctivitis in patients with PLG deficiency.

摘要

引言

本病例报告介绍了一名患木质化性结膜炎患者的临床、组织学和遗传学特征,并提供了一种长期治疗方案,即使用冷冻去除血浆输注联合局部用环孢素作为一种成功的治疗选择。

病例介绍

一名12岁的土耳其女孩多年来一直经历眼部瘙痒、刺激和光敏感度增加。初步临床检查发现双眼睑结膜有假膜和巨大乳头。从左眼取了一块膜进行组织学检查。自婴儿期以来严重合并症(先天性脑积水、哮喘、反复呼吸道感染和伤口愈合受损)的表现以及父母近亲结婚促使进行基因评估。假膜由纤维蛋白、肉芽组织和炎性细胞组成,符合木质化性结膜炎的诊断。基因分析显示纤溶酶原(PLG)存在致病性错义变异的纯合性,导致PLG缺乏。由于严重的全身并发症,患者通过冷冻去除血浆输注和环孢素滴眼液成功治疗,木质化性结膜炎完全缓解。

结论

本病例报告强调了在复发性慢性结膜炎患者中考虑PLG缺乏的重要性。冷冻去除血浆输注联合短期局部使用环孢素的长期治疗可能为PLG缺乏患者的木质化性结膜炎提供一种有效的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/52da/12331225/98036e9723d2/cop-2025-0016-0001-546953_F01.jpg

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