摘要
目的探讨IgG4相关性疾病的临床与病理学特点。方法对2例IgG4相关性疾病进行回顾,分析其临床表现、血清学、影像学及病理组织学特点,并文献复习。应用免疫组化染色进一步诊断并随访。结果例1因反复右上腹痛3月余,发现皮肤、巩膜黄染1周就诊。影像学示肝内、胰腺、双肾多发病变。血生化示血沉、球蛋白、总蛋白均明显高于正常值,血清IgG4水平为2 860 mg/L。例2为中老年女性,2例临床表现相似。2例镜下均表现为受累组织中纤维组织增生伴大量炎细胞浸润,炎细胞以浆细胞、淋巴细胞为主及较多的嗜酸性粒细胞,局部伴胶原化,间质小血管内皮细胞增生,部分呈闭塞性脉管炎改变。肝免疫组化示梭形细胞vimentin(+),CD21、desmin、S-100、CD34、CD1a、actin、bcl-2、HMB45、CD99和CK(-);浆细胞IgG4(+)(阳性细胞计数68个/HPF),IgG4/Ig G阳性细胞>70%。例2右上睑肿物免疫组化示浆细胞IgG4(+),且IgG4/Ig G阳性细胞>40%。结论 IgG4相关性疾病的临床表现与所受累的器官密切相关,当出现多器官受累及特征性的病理组织学形态时应首先考虑本病,进一步免疫组化检测可以基本确诊。糖皮质激素是目前公认的最有效的治疗方法。
Objective To study the clinicopathological features of IgG4-related diseases. Methods A retrospective study was performed to analyze the clinical,radiological,histopathological features in two cases of IgG4-related diseases,with review of the literature. Immunohistochemistry was used to further confirm the diagnosis with follow-up. Results The first patient was a middle-aged male. Because of right upper quadrant pain repeatedly for 3 months,the patient saw a doctor for yellowing of the skin and clera for one week. Imaging examination showed multiple lesions within the liver,pancreas and kidneys. Serological ESR,globulin,and total protein were significantly higher than normal,IgG4 levels in serum was 2860 mg / l by Elisa. Anoher patient was a quinquagenarian female. In the second case,histopatho Iogical findings included diffuse fibrosis with extensive Inflammatory cells, mainly plasma cells and lymphocytes, with eosinophilia. Small vascular endothelial cells showed hyperplasia and mesenchymal collagenization, even obliterative phlebitis. On immunohistochemical staining it showed that spindle cells were positive for vimetin,but negtive for CD21,desmin,S-100,CD34,CD1 a,Actin,bcl-2,HMB45,CD99 and CK. Plasma cells IgG4（ ＋）,the ratio of IgG4-positive plasma cells to Ig G-positive plasma cells was higher than 70%; the ratio of IgG4-positive plasma cells to Ig G-positive plasma cells was higher than 40% at the upper eyelid mass by immunohistochemical staining in the second patient.Results Clinical manifestations of IgG4-related disease is closely related to the affected organ. We should consider the disease firstly when patients have multiple organ involvements and characteristic histopathological features. Further immunohistochemical staining can basically confirmed the diagnosis. Glucocorticoids is internationally recognized as the most effective treatment method.
出处
《诊断病理学杂志》
CSCD
2015年第8期471-474,共4页
Chinese Journal of Diagnostic Pathology
