本文關(guān)鍵詞：IgG4相關(guān)性腦膜病變　出處：《中國(guó)現(xiàn)代神經(jīng)疾病雜志》2016年03期 　論文類型：期刊論文

  更多相關(guān)文章： 免疫球蛋白G 腦膜 病理學(xué) 免疫組織化學(xué)

【摘要】：目的探討Ig G4相關(guān)性腦膜病變的臨床病理學(xué)特征以及診斷與鑒別診斷要點(diǎn)。方法與結(jié)果男性患者,49歲,臨床表現(xiàn)為頭痛近2年并進(jìn)行性加重1月余,頭部MRI顯示左側(cè)頂葉占位性病變,增強(qiáng)掃描可見(jiàn)"腦膜尾征",手術(shù)完整切除病灶。組織學(xué)形態(tài),左側(cè)頂葉硬腦膜和腦實(shí)質(zhì)大量膠原纖維增生,其間散在灶狀細(xì)胞浸潤(rùn),多為較成熟的漿細(xì)胞,部分漿細(xì)胞內(nèi)可見(jiàn)勻質(zhì)紅染的Russell小體,其間散在淋巴細(xì)胞和少量嗜酸性粒細(xì)胞,局部可見(jiàn)小灶狀壞死,間質(zhì)纖維母細(xì)胞和小血管增生,未見(jiàn)包膜,病變累及周圍腦組織。免疫組織化學(xué)染色,漿細(xì)胞胞質(zhì)彌漫性表達(dá)Ig G和Ig G4(60%)、胞膜表達(dá)CD38和CD138,淋巴細(xì)胞胞膜表達(dá)CD3、CD4或CD20。血清Ig G4為1.05 g/L。最終病理診斷為(左側(cè)頂葉)Ig G4相關(guān)性腦膜病變可能性大。術(shù)后予抗感染、抗癲、營(yíng)養(yǎng)支持治療,癥狀明顯好轉(zhuǎn),出院后未按醫(yī)囑定期隨訪。結(jié)論 Ig G4相關(guān)性腦膜病變臨床少見(jiàn),且缺乏典型臨床表現(xiàn)和特征性影像學(xué)改變,術(shù)前診斷與鑒別診斷困難,血清Ig G4水平升高是其診斷的重要線索,明確診斷仍需依靠特征性的組織學(xué)形態(tài)和免疫組織化學(xué)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.

【作者單位】： 南方醫(yī)科大學(xué)基礎(chǔ)醫(yī)學(xué)院病理學(xué)系;南方醫(yī)科大學(xué)南方醫(yī)院病理科;
【基金】：廣東省廣州市科技計(jì)劃項(xiàng)目(項(xiàng)目編號(hào):2014Y2-00142)~~
【分類號(hào)】：R593.2;R747.9
【正文快照】： Ig G4相關(guān)性疾病是新近定義的自身免疫性疾射、調(diào)節(jié)反射和輻輳反射靈敏,鼻唇溝對(duì)稱,伸舌居病類型,最初被Sarles等[1]報(bào)告為自身免疫性胰腺中。腦神經(jīng)檢查未見(jiàn)異常。腦膜刺激征陰性,病理炎(AIP),病理學(xué)檢查可見(jiàn)胰腺系統(tǒng)性硬化。后續(xù)征未引出。四肢肌力和肌張力無(wú)明顯異常。研究，

本文編號(hào)：1420142