IgG4相关肝病临床病理及预后特征分析  

IgG4-related liver disease: clinicopathological characteristics and prognosis

作　　者：王欣欣[1] 王泰龄[2] 孔贺利[1] 孙琳[1] 吕福东[1] 

机构地区：[1]首都医科大学附属北京佑安医院病理科,北京100069 [2]北京中日友好医院病理科,北京100029

出　　处：《诊断病理学杂志》2014年第1期8-12,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨IgG4相关肝病的临床病理特征及预后。方法收集2例IgG4相关肝病病例的临床资料。行常规HE染色和特殊化学染色,光镜下观察肝活检组织形态特点,并结合CD138、MUM-1和IgG、IgG4免疫组化检测进行分析。治疗后进行随访。结果 2例患者临床表现为转氨酶升高、血清总球蛋白及免疫球蛋白G显著升高。肝组织中大量成熟浆细胞、淋巴细胞浸润,重度肝细胞坏死;免疫组化IgG4阳性浆细胞增多(>10个/HPF)。类固醇治疗后,转氨酶降至正常,血清球蛋白和IgG明显下降。结论 IgG4相关肝病是IgG4相关系统性疾病的表现形式之一,病变表现形式重,类固醇治疗效果理想,预后好。Objective To explore the clinical and pathological features and prognosis of IgG4-related liver disease through clinical cases and literature reviews. Methods Two cases of IgG4-related liver disease patients were analyzed. The clinical data, including serological and image changes were collected. The clinicopathologic characteristics were evaluated by morphology and immunohistochemistry for CD138, MUM-1, IgG and IgG4. The cases were then followed-up after treatment. Results The serum transaminase, globulin and IgG significantly increased in two cases; Liver biopsy showed a large number of mature plasma cells, lymphocytes infiltration, severe liver cell necrosis and IgG4-positive plasma cells were increased （ 〉 10/HPF ） ; transaminase returned to normal, serum globulin and IgG decreased after steroid treatment. Conclusion IgG4-related liver disease is a part of systemic IgG4-related diseases, although the tissue damage shows serious, response to steroid therapy is high, with a good prognosis.

关 键 词：IgG4相关肝病 临床 病理 预后 

分 类 号：R575[医药卫生—消化系统]