IgG4相关性疾病临床病理学特征分析

Analysis of clinicopathological characters for IgG4-related disease

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En Vision两步法染色Ig G4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。

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, lmmunohistoehemieal staining re- suits 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.