以OMBA为首发症状的轻链型淀粉样变性1例

doi:10.13701/j.cnki.kqyxyj.2025.10.013

口腔医学研究 ›› 2025, Vol. 41 ›› Issue (10): 912-915.DOI: 10.13701/j.cnki.kqyxyj.2025.10.013

以OMBA为首发症状的轻链型淀粉样变性1例

张静睿^1,2, 孟鑫^1,2, 马俊^1,2, 李欣雨^1,2, 徐柯², 王买全², 蔺栋鹏², 王永功^2*

1.河南大学口腔医学院河南开封 475001;
2.河南省人民医院口腔颌面外科河南郑州 450003

收稿日期:2024-12-27 发布日期:2025-10-23
通讯作者: *王永功,E-mail:wygkyz@163.com
作者简介:张静睿(1998~ ),男,河南鹤壁人,硕士在读,研究方向:口腔颌面部肿瘤、颞下颌关节紊乱、口腔恶性肿瘤、口腔黏膜病。
基金资助:
国家重点研发计划(项目编号:2018YFC0114505)

AL Amyloidosis with Oral Mucosal Bullous Lesions as Initial Symptom: A Case Report

ZHANG Jingrui^1,2, MENG Xin^1,2, MA Jun^1,2, LI Xinyu^1,2, XU Ke², WANG Maiquan², LIN Dongpeng², WANG Yonggong^2*

1. Henan University School of Stomatology, Kaifeng 475001, China;
2. Department of Oral and Maxillofacial Surgery, Henan Provincial People's Hospital, Zhengzhou 450003, China

Received:2024-12-27 Published:2025-10-23

摘要/Abstract

摘要： 系统性轻链型(systemic light-chain,AL)淀粉样变性(amyloidosis)是一种与克隆性浆细胞异常增殖相关的恶性疾病,其特征是产生错误折叠和不稳定的免疫球蛋白轻链,导致多系统淀粉样蛋白沉积,进而发展为器官功能障碍并最终衰竭。口腔黏膜大疱样淀粉样变性(oral mucosal bullous amyloidosis,OMBA)是指淀粉样变性累及皮肤及口腔黏膜,表现为大疱样病损的一种罕见临床表现,通常以吞咽困难为首发症状,且容易与口腔大疱样黏膜病混肴,导致误诊或漏诊。本文报告了河南省人民医院口腔颌面外科诊治的1例以口腔黏膜大疱样病损为主要表现的AL型淀粉样变性1例,并结合文献讨论其临床特点、诊断及治疗策略,旨在提高口腔科医师对这一罕见病症的认识,减少漏诊和误诊的发生。

关键词: 轻链型淀粉样变性, 口腔黏膜大疱样淀粉样变性, 诊断策略

Abstract: Systemic light-chain (AL) amyloidosis is a malignant disorder associated with clonal plasma cell dyscrasia. It is characterized by the production of misfolded and unstable immunoglobulin light chains, leading to multisystem amyloid protein deposition, progressive organ dysfunction, and eventual failure. Oral mucosal bullous amyloidosis (OMBA) refers to a rare clinical manifestation of amyloidosis involving the skin and oral mucosa, presenting as bullous lesions. OMBA often initially manifests as dysphagia and is easily confused with other bullous mucosal diseases, resulting in misdiagnosis or missed diagnosis. This report presents a case of AL amyloidosis with OMBA as the primary clinical feature, diagnosed and managed at the Department of Oral and Maxillofacial Surgery, Henan Provincial People's Hospital. Combined with a review of the literature, the report discusses the clinical characteristics, diagnostic approaches, and treatment strategies of this rare condition. The aim is to enhance awareness of this disease among dental practitioners and reduce the incidence of misdiagnosis and missed diagnosis.

Key words: systemic light chain amyloidosis, oral mucosal bullous amyloidosis, diagnostic strategies

张静睿, 孟鑫, 马俊, 李欣雨, 徐柯, 王买全, 蔺栋鹏, 王永功. 以OMBA为首发症状的轻链型淀粉样变性1例[J]. 口腔医学研究, 2025, 41(10): 912-915.

ZHANG Jingrui, MENG Xin, MA Jun, LI Xinyu, XU Ke, WANG Maiquan, LIN Dongpeng, WANG Yonggong. AL Amyloidosis with Oral Mucosal Bullous Lesions as Initial Symptom: A Case Report[J]. Journal of Oral Science Research, 2025, 41(10): 912-915.

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以OMBA为首发症状的轻链型淀粉样变性1例

AL Amyloidosis with Oral Mucosal Bullous Lesions as Initial Symptom: A Case Report

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