特发性肥厚性硬脑膜炎临床病理分析  

Clinical and pathological analysis of idiopathic hypertrophic pachymeningitis

作　　者：王蔚[1] 陈晓东[1] 彭大云[1] 赖续文[1] 周永梅[1] 崔静[2] 

机构地区：[1]广州军区广州总医院病理科,510010 [2]广州军区广州总医院磁共振室,510010

出　　处：《中国微侵袭神经外科杂志》2016年第2期60-62,共3页Chinese Journal of Minimally Invasive Neurosurgery

基　　金：广东省医学科研基金(编号:2013037)

摘　　要：目的探讨特发性肥厚性硬脑膜炎(idiopathic hypertrophic pachymeningitis,IHP)的病理诊断要点。方法回顾性分析3例IHP病人的临床和影像学资料,显微镜下观察其组织学形态。男性2例,女性1例,临床表现为头痛、腰背部疼痛伴双下肢麻木、乏力,均行手术切除。结果影像学显示颅内或椎管内硬膜弥漫增厚或呈结节状。组织学:病变内纤维组织显著增生、胶原化,大量淋巴细胞、浆细胞浸润,淋巴滤泡形成,伴有多少不等的中性粒细胞聚集。免疫表型:增生的纤维组织SMA、MSA阳性;浆细胞CD38、CD138、EMA和Ig G阳性,但Ig G4阴性;CD45RO阳性T淋巴细胞明显多于CD20阳性B淋巴细胞。结论 IHP少见,其正确诊断需结合临床和影像学特征以及病理形态综合分析。Objective To investigate the key points for pathological diagnosis of idiopathic hypertrophic pachymeningitis（IHP）.Methods The clinical and imaging data of 3 patients with IHP undergoing surgery were analyzed retrospectively. The morphological characteristics were observed under light microscope. The 2 male and 1 females undergoing surgery presented common complaints of headache or lower back pain with lower extremity numbness and weakness. Results Imaging showed diffuse or nodular thickening of the dura. Microscopically, the thickening of the dura was associated with dense fibrosis and collagen deposition, accompanyed with a mixed inflammatory infiltrate composed mainly of plasma cells and small lymphocytes. Neutrophils can be seen in some cases.Immunohistochemistry showed fibrous tissue positive for SMA and MSA. The plasma cells were positive for CD38, CD138, EMA and Ig G, but negative for Ig G4. The CD45RO-positive T lymphocytes were markedly more than CD20-positive B lymphocytes. Conclusions IHP is rare and its accurate diagnosis depends on the comprehensive analysis of clinical history, imaging and pathological features.

关 键 词：硬脑膜炎 肥厚性 特发性 病理学 临床 影像学 

分 类 号：R742.9[医药卫生—神经病学与精神病学]