Thymomatous Myasthenia Gravis

研究195例重症肌無力症病人，其中90例接受胸線摘除術治療，檢查胸線之病理變化，發現30例〈33.3%〉為胸線腫瘤，胸線腫瘤性重症肌無力症病人發病年齡較晚，平均為42.2±14.6歲；男女性別比率均等；臨床症狀較嚴重，多屬於全身性Ⅱb，Ⅲ或型。胸線腫瘤可由放射線檢查發現，特爺是胸腔電腦斷層攝影之診斷率高達84.2%其餘病人由於胸線腫瘤小，經開刀後病理切片才確定。胸線瘤大多屬於混合淋巴球及表皮細胞型〈80.0%〉，其他為惡性胸線瘤〈13.3%〉或脂肪胸線瘤〈6.7%〉。胸線之組織型態與症狀嚴重度無關，合併胸線瘤之重症肌無力症出現抗橫紋肌抗體，抗核酸抗體及乙型肝炎抗原之比率比無胸線瘤群為高；HLA則除有較高之BW62外，無其他phenotype之差異，近年來追蹤研究顯示除了惡性胸線腫瘤造成之死亡率〈6.9%〉外，合併胸線腫瘤重症肌無力症病人之預後與無瘤群相同，開刀後症中寬姐及無症中比率達46.6%。預後的改善可能歸功於近年來胸線摘除開刀技術及呼吸照顧之進步。

關鍵字

無資料

並列摘要

The clinical course of 195 cases of myasthenia gravis was studied. Among them, 90 myasthenia patients received thymectomies, and of these, thirty cases of thymoma were pathologically proven. The incidence of thymomatous myasthenia gravis in these thymectomized patients was 33.3%. The average age of these 30 cases was 44.4±14.2 years and the sex ratio was almost equal. Their onset age was at 42.2±14.6 years. Before thymectomy, the clinical severity of 89.7% of thymoma patients were of a generalized type, especially typeⅡb, Ⅲ or Ⅳ. Compared with the two groups of thymic hyperplasia and hypoplasia, thymoma patients had a later onset with an equal sex distribution, and manifested a more severe myasthenia. The thymoma may be detected on radiological examination of the chest, especially by mediastinal CT scan. But in 16% of cases, the tumors were so small that they were found only after thymectomy. All patients underwent maximal thymectomy. The pathology of the thymus in these patients was predominantly of the lymph-epithelial type. Four cases (13.3%) were malignant thymoma and received radiation therapy after thymectomy. The histology of the tumor seemed to have no relationship to the severity of the myasthenia gravis. On follow-up of the showed improvement; 13.8% were in the state of remission; 65.5% showed improvement; 13.3% were unchanged and the mortality rate was 6.8%. Postoperative improvement was either immediate or gradual.

並列關鍵字

thymoma ； myasthenia gravis

國際替代計量

Thymomatous Myasthenia Gravis

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