中文摘要：

目的：阐述Tfh细胞及相关分子在重症肌无力发病中的作用。通过分析Tfh细胞及相关分子在眼肌型和全身型重症肌无力患者外周血的表达差异,阐明眼肌型和全身型重症肌无力患者外周发生了不同的体液免疫反应;在描述重症肌无力患者症状严重程度方面,比较绝对评分和QMG评分的优劣性。方法：运用抗体标记Tfh细胞相关分子CXCR5、ICOS、Bcl-6及CXCL13,应用流式细胞术检测、分析这些分子在非重症肌无力、眼肌型和全身型重症肌无力患者外周血的表达;对病情做QMG评分和绝对评分,分析其与CXCL13表达的相关性。结果：在眼肌型、全身型重症肌无力患者外周血中,CXCR5、ICOS和Bcl-6分子在CD4＋T细胞的表达增高,其中CXCR5、Bcl-6分子表达在3组之间的差异存在统计学意义;随MG病情发展,外周血CXCL13的表达上调,CXCL13与QMG评分和绝对评分相关系数分别为0.669和0.797。结论：Tfh细胞及相关分子CXCR5、Bcl-6、CXCL13的表达参与了MG的发生、发展过程;Tfh细胞及相关分子在全身型重症肌无力患者外周血的表达高于眼肌型重症肌无力,这是全身型重症肌无力患者临床症状更重和症状评分更高的原因,也是全身型重症肌无力患者外周血抗体阳性率高于眼肌型重症肌无力的原因;在重症肌无力患者病情评测中,绝对评分较QMG评分更加准确,与临床症状的相关性更高。

英文摘要：

Objective： To elaborate the role of Tfh cells and molecules in the pathogenesis of myasthenia gravis By analyzing the differential expression of Tfla cells and molecules in ocular and generalized myasthenia gravis patients peripheral blood, the humoral immune response is clarified between ocular and generalized myasthenia gravis patients in peripheral; Comparing the absolute scores and QMG scores on description of myasthenia gravis symptom severity. Methods： Using antibodies to marker the molecules, CXCR5, ICOS, Bcl-6 and CXCL13. Detected by flow cytometry,analysis of differential expression of these molecules in non-MG, ocular and generalized myasthenia gravis patients peripheral blood. Results： CXCR5, ICOS and Bcl-6 increased expression in CD4＋ T cells from ocular and generalized myasthenia gravis patients in peripheral blood, expression of CXCR5 and Bcl-6 have a statistical significance among the three groups. Conclusion＂ Tfla cells and the molecules, CXCR5, Bcl-6, CXCL13 were involved in the occurrence and development of the MG; The expression of Tfla cells and molecules in peripheral blood from patients with generalized myasthenia gravis was higher than that of patients with ocular myasthenia gravis. That is why generalized myasthenia patients have more serious symptoms, higher scores, and higher positive rate of antibody than ocular myasthenia gravis patients. On evaluation of the MG patient＇s condition, the absolute scores had a advantage to the QMG scores, absolute scores was more accurate, and more relevant to clinical symptoms.