IgG4相关性脑膜病变

发布时间：2018-01-13 19:08

  本文关键词：IgG4相关性脑膜病变　出处：《中国现代神经疾病杂志》2016年03期 　论文类型：期刊论文

  更多相关文章： 免疫球蛋白G 脑膜 病理学 免疫组织化学

【摘要】：目的探讨Ig G4相关性脑膜病变的临床病理学特征以及诊断与鉴别诊断要点。方法与结果男性患者,49岁,临床表现为头痛近2年并进行性加重1月余,头部MRI显示左侧顶叶占位性病变,增强扫描可见"脑膜尾征",手术完整切除病灶。组织学形态,左侧顶叶硬脑膜和脑实质大量胶原纤维增生,其间散在灶状细胞浸润,多为较成熟的浆细胞,部分浆细胞内可见匀质红染的Russell小体,其间散在淋巴细胞和少量嗜酸性粒细胞,局部可见小灶状坏死,间质纤维母细胞和小血管增生,未见包膜,病变累及周围脑组织。免疫组织化学染色,浆细胞胞质弥漫性表达Ig G和Ig G4(60%)、胞膜表达CD38和CD138,淋巴细胞胞膜表达CD3、CD4或CD20。血清Ig G4为1.05 g/L。最终病理诊断为(左侧顶叶)Ig G4相关性脑膜病变可能性大。术后予抗感染、抗癫、营养支持治疗,症状明显好转,出院后未按医嘱定期随访。结论 Ig G4相关性脑膜病变临床少见,且缺乏典型临床表现和特征性影像学改变,术前诊断与鉴别诊断困难,血清Ig G4水平升高是其诊断的重要线索,明确诊断仍需依靠特征性的组织学形态和免疫组织化学表型。
[Abstract]:Objective to study the characteristics and clinical pathological diagnosis and differential diagnosis of meningeal lesions. Ig G4 correlation between male patients, methods and results of 49 years, nearly 2 years of clinical manifestations were headache and progressive increase in more than 1 months, the head MRI showed left parietal lobe lesions, enhanced scan showed dural tail sign, complete resection lesions. Histological morphology, left parietal dura and brain parenchyma collagenous fiber hyperplasia, scattered infiltration in focal cells, more mature plasma cells, plasma cells seen in homogeneous red stained Russell bodies, scattered eosinophils in lymphocytes and a small amount of visible local small focal necrosis, between interstitial fibroblasts and vascular proliferation, no capsule, brain tissue surrounding lesions. Immunohistochemical staining, plasma cell cytoplasm diffuse expression of Ig G and Ig G4 (60%), the expression of CD38 and CD138 in cell membrane, cell membrane The expression of CD3, CD4 or CD20. Ig G4 serum was 1.05 g/L. for the final pathological diagnosis (left parietal lobe) Ig G4 associated meningeal lesions possibility. After treated with anti infection, anti epilepsy, nutritional support treatment, symptoms improved significantly, as the doctor discharged without regular follow-up. Conclusion Ig G4 rare associated brain membrane lesions and clinical. The lack of typical clinical manifestations and characteristic imaging changes, preoperative diagnosis and differential diagnosis of difficult, elevated serum Ig G4 level is an important clue to the diagnosis, the diagnosis still need to study the morphologic and immunohistochemical phenotype depends on the characteristic of the organization.

【作者单位】： 南方医科大学基础医学院病理学系;南方医科大学南方医院病理科;
【基金】：广东省广州市科技计划项目(项目编号:2014Y2-00142)~~
【分类号】：R593.2;R747.9
【正文快照】： Ig G4相关性疾病是新近定义的自身免疫性疾射、调节反射和辐辏反射灵敏,鼻唇沟对称,伸舌居病类型,最初被Sarles等[1]报告为自身免疫性胰腺中。脑神经检查未见异常。脑膜刺激征阴性,病理炎(AIP),病理学检查可见胰腺系统性硬化。后续征未引出。四肢肌力和肌张力无明显异常。研究，

本文编号：1420142