重症肌无力伴脂肪胸腺瘤的临床病理特征、诊断和治疗(附3例报告)  

Characteristic. diagnosis and treatment of myasthenia gravis complicated with lipothymoma: A report of 3 cases

作　　者：涂来慧[1] 吴涛[1] 蒋建明[1] 张仁琴[1] 丁素菊[1] 蔡建英[1] 

机构地区：[1]第二军医大学长海医院神经内科,上海200433

出　　处：《第二军医大学学报》2003年第11期1178-1179,共2页Academic Journal of Second Military Medical University

摘　　要：报告3例重症肌无力伴脂肪胸腺瘤的中老年患者临床资料,其肌无力症状均严重,2例发生危象。手术切除胸腺瘤,疗效均显著,其中2例已痊愈。手术病理证实瘤体都由大量成熟脂肪细胞、极少胸腺上皮瘤细胞和淋巴细胞组成,呈微小状分布,易漏诊。病情严重程度与胸腺瘤细胞数目不成正比,而与胸腺病理特征和特异性免疫应答相关。根据肌无力病史,检测血清中烟碱型乙酰胆碱受体抗体(nAchR-Ab)、骨骼肌柠檬酸提取物抗体(CAE-Ab)、重复神经剌激肌电图(RNS-EMG),纵隔CT或MRI扫描,可早期诊断;手术病理检查并作免疫组化染色可确诊。提示须重视脂肪胸腺瘤富含脂肪、隐藏微小上皮细胞瘤的临床病理特征,避免漏诊。The data of 3 patients with myasthenia gravis complicated with lipothymoma were reviewed. All the 3 patients were severe and 2 had myasthenia crisis. Two patients were cured after thymectomy. Pathology found that the lipothymoma was comprised of many mature fatty cells, few thymic epithelial tumor cells and lymphocytes, which were distributed in a micro-island shape and easy to be neglected. The severity of the disease was not related to the number of thymic epithelial tumor cells; the pathological characteristic was related to the specific immune response. Early diagnosis could be made based on the history of myasthenia gravis, detection of serum nAChR-Ab, CAE-Ab, RNS-EMG levels, mediastinum CT scanning and MRI. And the diagnosis could be confirmed by pathological examination. It is indicated that special attention should be paid to the clinico-pathological characteristics of lipothymoma to avoid misdiagnosis.

关 键 词：重症肌无力 脂肪胸腺瘤 病理特征 诊断 治疗 并发症 

分 类 号：R746.1[医药卫生—神经病学与精神病学] R736.3[医药卫生—临床医学]