类鼻咽恶性肿瘤表现的IgG4相关性疾病  

IgG4-related disease with nasopharyngeal malignancy-like manifestations

作　　者：林惜君 林沛亮 刘翔[1] LIN Xijun;LIN Peiliang;LIU Xiang(Department of Otolaryngology Head and Neck Surgery,Sun Yat-sen Memorial Hospital,Sun Yat-sen University,Guangzhou,510000,China)

机构地区：[1]中山大学孙逸仙纪念医院耳鼻咽喉头颈外科,广州510000

出　　处：《临床耳鼻咽喉头颈外科杂志》2022年第4期293-296,共4页Journal of Clinical Otorhinolaryngology Head And Neck Surgery

摘　　要：目的:探讨IgG4相关疾病(IgG4-RD)的临床特征和影像学特征,提高鼻咽IgG4-RD早期的诊断能力,减少误诊与漏诊。方法:通过电子病例系统采集患者基本信息,包括年龄、性别、症状、病程和诊疗过程。记录患者住院期间及门诊随诊的实验室检查资料,包括鼻内镜检查、EB病毒水平、IgG4水平及C反应蛋白水平等。对患者的CT、MRI以及PET-CT特征进行总结分析,并收集所有患者的病理和免疫组织化学结果。结果:4例患者均行病灶部分切除活检术,病理结果显示炎性肉芽组织和纤维组织增生,淋巴细胞、浆细胞和中性粒细胞浸润较多,免疫组织化学IgG4+浆细胞每高倍视野均>10个。结合病史、影像学检查、血清学结果及相关治疗,最终诊断为IgG4-RD,经激素和免疫抑制治疗后症状均有明显改善。结论:IgG4-RD与鼻咽癌的临床表现高度相似,对伴有似鼻咽癌症状且多次活检无法明确病理的患者,需要与IgG4-RD进行鉴别,及时诊断IgG4-RD对预防活动性疾病患者继发性脏器损害具有重要意义。Objective:The purpose of this article was to discuss the clinical features and imaging characteristics of IgG4-related disease(IgG4-RD)in order to identify nasopharyngeal IgG4-RD at an early stage.Methods:The basic information of the patients,including age,sex,symptoms,disease duration and treatment process,was collected through the electronic case system.Laboratory tests including nasal endoscopy,EBV levels,IgG4 levels and C-reactive protein levels were recorded during hospitalization and outpatient follow-up.All radiological imaging and postoperative pathology data are collected,analyzed and summarized.Results:All patients underwent partial excisional biopsy of the lesion.The pathological findings showed inflammatory granulomatous and fibrous tissue hyperplasia with a high infiltration of lymphocytes,plasma cells and neutrophils,and immunohistochemistry examination showed IgG4+plasma cells were more than 10 per high magnification field.Combining medical history,imaging,serological findings and relevant treatment,all four patients were diagnosed with IgG4-associated disease.And their symptoms improved significantly after hormonal and immunosuppressive treatment.Conclusion:IgG4-RD has a highly similar clinical presentation with nasopharyngeal carcinoma.Differentiation from IgG4-RD should be considered for those pathology cannot be clarified by multiple biopsies.Timely diagnosis of IgG4-RD is important to prevent secondary organ damage in patients with active disease.

关 键 词：IgG4相关疾病 鼻咽肿瘤 诊断 

分 类 号：R739.63[医药卫生—肿瘤]