IgG4相关硬化性胰腺炎2例临床病理观察

IgG4-related pancreas sclerosing disease: a clinicopathologic analysis of 2 cases

目的探讨IgG4相关硬化性胰腺炎的临床病理学特征、诊断及鉴别诊断。方法对2例IgG4相关硬化性胰腺炎进行HE染色、免疫组化染色、光镜观察,并复习相关文献。结果 HE染色病变胰腺组织中显示胰腺腺泡的萎缩和破坏,纤维组织增生,小叶间质席纹状硬化,并伴有淋巴组织增生、大量成熟的浆细胞浸润及闭塞性静脉炎。免疫表型:浆细胞呈IgG、IgG4(+),该2例浆细胞IgG4计数平均为121个/HPF和84个/HPF,IgG4(+)/IgG(+)比值分别为40%及47%。结论 IgG4相关硬化性胰腺炎是IgG4相关硬化性疾病的一部分,主要累及胰腺,也可累及胆管、泪腺、涎腺、乳腺、甲状腺等器官,病理诊断需与胰腺的炎性假瘤、局灶性淋巴组织增生、原发性硬化性胆管炎相鉴别。

Objective To study the clinical and histopathologic features,diagnosis and differential diagnosis of IgG4-related pancreatic sclerosing disease. Methods Two cases of IgG4-related pancreas sclerosing disease were studied by microscopy,immunohistochemisty with review of related literatures. Results The results of HE staining showed fibrous hyperplastic lesions of pancreatic tissue,lymphoid hyperplasia,with a large amount of mature plasma cell infiltration,and obliterative phlebitis,accompanied by pancreatic acinar atrophy and destruction; interlobular stroma showed storiform hardening. The immune phenotyping showed that plasma cells were IgG and IgG4 positive,the two cases of IgG4 positive plasma cell count was 121 / HPF and 84 / HPF,IgG4 ＋ / IgG ＋ ratios were 40% and 47%. Conclusion IgG4 related sclerosing pancreatitis is a part of IgG4 related sclerosing disease,mainly involving the pancreas; it can also affect bile duct,lacrimal gland, thyroid, breast, and other organs. The differential diagnosis from pancreatic inflammatory pseudotumor,focal lymphoid hyperplasia and primary sclerosing cholangitis should be considered.