慢性特发性轴索性多神经病的病理及免疫组化研究

Pathological and immunohistochemical studies in chronic idiopathic axonal polyneuropathy patients

目的研究慢性特发性轴索性多神经病(chronic idiopathic axonal polyneuropathy,CIAP)病理改变特点,并探索腓肠神经炎细胞CD3、CD20、CD68抗体及其微小血管内皮细胞膜结合性血栓调节蛋白(thrombomodulin,TM)、内皮源性一氧化氮合酶(endothelial-nitricoxide synthase,e NOS)的表达规律。方法 10例经过临床、电生理、腓肠神经活检病理检查证实的CIAP患者,均进行腓肠神经活检标本的常规病理组织学染色以及以抗CD3、CD20、CD68、TM、e NOS、v WF(von Willebrand factor,v WF)抗体作为第一抗体的免疫组织化学染色。结果 10例患者腓肠神经病理检查显示有髓神经纤维轻-中度减少,伴随轴索变性和再生,部分可见轻微脱髓鞘改变,4例患者出现毛细血管基底膜肥厚。4例患者腓肠神经神经束衣间小血管周围有散在分布的CD68阳性单核细胞浸润。所有患者血管内皮细胞v WF、e NOS、TM均正常表达。结论 CIAP病理特点为轴索损害为主,发病可能和体液免疫异常有关,部分患者毛细血管基底膜肥厚提示血管内皮细胞可能受损,但内皮细胞功能相关蛋白表达初步提示正常。

Objective To investigate the pathological features of chronic idiopathic axonal polyneuropathy,and the expression of endothelial-nitricoxide synthase and thrombomodulin in the endothelium of the vascular in sural nerve in patients with CIAP. Methods We collected 10 patients with CIAP confirmed by clinical and electrophysiological examination and nerve pathologydiagnosis. Sural nerve biopsies were performed in all of them. We did the routine histological staining and Envision immunohistochemical staining with first antibodies against inflammatory cell markers of CD3,CD20,CD68 and endothelial cell markers of e NOS,TM and v WF. Results Sural nerve biopsies showed mild to moderate loss of myelinated fibers with axonal degeneration and regeneration. A few of them showed slight demyelination of myelinated fibers and thick basal membrane of capillary. 4 patients had CD68-positive mononuclear cells within the fascicular. No CD3 and CD20-positive lymphocytes were found. All of the patients had positive expression with e NOS,TM,v WF in the endothelial cells of the blood vessels. Conclusion The main pathological features were axonal damage. The pathogenesis of CIAP might relate to humoral immunological abnormalities. Thick basal membrane of capillary in some patients indicated the vascular endothelial cell functions injured,but the expression of vascular endothelial cell functional proteins was normal.