自身免疫性肝炎：胸腺瘤的一种罕见表现。
Autoimmune hepatitis: an uncommon presentation of thymoma.
作者信息
Mendogni Paolo, Rosso Lorenzo, Tosi Davide, Palleschi Alessandro, Righi Ilaria, Minonzio Francesca, Fusco Nicola, Nosotti Mario
机构信息
Thoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan - Italy.
Department of Internal Medicine, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan - Italy.
出版信息
Tumori. 2016 Nov 11;102(Suppl. 2):E18432F3-EDD2-4780-AE3C-D9153373E123. doi: 10.5301/tj.5000505.
PURPOSE
In a substantial proportion of patients with thymoma, many different types of paraneoplastic syndromes are observed. The association between thymoma and autoimmune liver diseases, however, has been found in very few cases.
METHODS
We report the case of a 31-year-old man affected by autoimmune hepatitis associated with myasthenia gravis and thymoma, successfully treated with extended thymectomy.
RESULTS
The patient is free from neoplastic and hepatic disease 4 years after surgery. Eighteen months after thymectomy, an exacerbation of hepatitis was successfully treated with steroids.
CONCLUSIONS
To the authors' knowledge, only 7 cases of myasthenia gravis associated with thymoma and autoimmune hepatitis have been reported in the English-language literature. The exact role of thymoma in immune-mediated hepatitis is unclear. It seems likely that thymoma-associated T-cell abnormalities, due to the presence of thymoma, may have a role in the development of this rare clinical triad of autoimmune hepatitis, thymoma and myasthenia gravis.
目的
在相当一部分胸腺瘤患者中，可观察到多种不同类型的副肿瘤综合征。然而，胸腺瘤与自身免疫性肝病之间的关联仅在极少数病例中被发现。
方法
我们报告一例31岁男性患者，患有与重症肌无力和胸腺瘤相关的自身免疫性肝炎，经扩大胸腺切除术后成功治愈。
结果
患者术后4年无肿瘤及肝脏疾病。胸腺切除术后18个月，肝炎加重，经类固醇治疗成功。
结论
据作者所知，英文文献中仅报道了7例与胸腺瘤和自身免疫性肝炎相关的重症肌无力病例。胸腺瘤在免疫介导性肝炎中的确切作用尚不清楚。由于胸腺瘤的存在，胸腺瘤相关的T细胞异常似乎可能在这种罕见的自身免疫性肝炎、胸腺瘤和重症肌无力临床三联征的发生中起作用。
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