CAJ | 학술논문

目的：探讨IgG4相关性疾病的病理学形态、免疫表型特征。方法观察12例IgG4相关性疾病的镜下特点，结合免疫组化EnVision两步法染色检测IgG、IgG4、CD138、CD34的表达，分析IgG4相关性疾病的病理学形态特征。结果 IgG4相关性疾病主要表现为组织弥漫性纤维化；伴大量淋巴细胞、浆细胞浸润，围绕血管神经分布；闭塞性静脉炎形成，免疫组化EnVision两步法染色IgG4阳性浆细胞与IgG阳性浆细胞比例>40%。结论 IgG4相关性疾病临床特点和影像学无特异性，易误诊为肿瘤，术前血清IgG4检测可作为疑似病例的首选方法。
목적：탐토IgG4상관성질병적병이학형태、면역표형특정。방법관찰12례IgG4상관성질병적경하특점，결합면역조화EnVision량보법염색검측IgG、IgG4、CD138、CD34적표체，분석IgG4상관성질병적병이학형태특정。결과 IgG4상관성질병주요표현위조직미만성섬유화；반대량림파세포、장세포침윤，위요혈관신경분포；폐새성정맥염형성，면역조화EnVision량보법염색IgG4양성장세포여IgG양성장세포비례>40%。결론 IgG4상관성질병림상특점화영상학무특이성，역오진위종류，술전혈청IgG4검측가작위의사병례적수선방법。
Purpose To study the clinicopathological features and immunohistochemical characteristics of IgG4-related disease. Meth-ods The microscopic characteristics and immunohistochemical staining (EnVision) of IgG, IgG4, CD138 and CD34 have been per-formed on 12 cases of IgG4-related disease. Results IgG4-related disease were characterized by diffuse fibrosis, accompanied with in-filtrating of dense lymphocytes and plasma cells surrounding neurovascular and occlusive phlebitis. Immunohistochemical staining re-sults showed the ratio of IgG4+/IgG+ cells were over 40%. Conclusions IgG4-related disease is absent of characteristic clinical and radiographic features and is easily misdiagnosed as tumor. Preoperative serum IgG4 detection could be used as the prior examina-tion for the suspected cases.