Santos Ernestina, Silva Ana Martins, Stroebel Philipp, Marinho Antonio, Willcox Nick, Goncalves Guilherme, Lopes Carlos, Marx Alexander, Leite Maria Isabel
Departments of Neurology (ES, AMS) and Pathology (CL), Clinical Immunology (A. Marinho) Hospital Santo Antonio/ Centro Hospitalar Universitario do Porto; Instituto de Ciencias Biomedicas de Abel Salazar (ES, AMS, GG), University of Porto, Portugal; Institute of Pathology (PS), University Medical Center Göttingen, University of Göttingen, Germany; Neurosciences Group (NW, MIL), Nuffield Department of Clinical Neurology, Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, UK; and Institute of Pathology (A. Marx), University Medical Centre Mannheim, University of Heidelberg, Mannheim, Germany.
Neurol Clin Pract. 2019 Feb;9(1):48-52. doi: 10.1212/CPJ.0000000000000551.
Thymomas appear very rarely after extended thymectomy for early-onset myasthenia gravis (EOMG). We describe 2 such cases that highlight potential early warning signs.
In their 20s, one woman and one man developed EOMG (AChR antibody-positive), requiring extended transsternal removal of hyperplastic thymi at ages 35 and 27, respectively. Their myasthenia gravis was readily controlled for the next 10 and 7 years before deteriorating in both, with appearance of late clinical features and anticytokine autoantibodies suggesting underlying thymomas, namely respiratory infections, genital herpes, chronic candidiasis, and alopecia in the woman and erythroderma and lichen planus in the man, followed by , , and cytomegalovirus infections plus chronic hepatitis during intensifying immunosuppressive therapy. Type B thymomas were then detected. Despite surgery or radiotherapy, and intensive drug therapy, the patients died 7 and 1 years later.
Certain infections/dermatologic manifestations that associate with long-standing thymomas may herald their late appearance, despite previous thymectomy.
在因早发型重症肌无力（EOMG）接受扩大胸腺切除术后，胸腺瘤非常罕见。我们描述了2例此类病例，突出了潜在的早期预警信号。
一名女性和一名男性在20多岁时患上EOMG（乙酰胆碱受体抗体阳性），分别在35岁和27岁时接受了经胸骨扩大切除增生胸腺的手术。在接下来的10年和7年里，他们的重症肌无力很容易得到控制，之后两人的病情均恶化，出现了晚期临床特征和抗细胞因子自身抗体，提示存在潜在的胸腺瘤，即该女性出现呼吸道感染、生殖器疱疹、慢性念珠菌病和脱发，该男性出现红皮病和扁平苔藓，随后在强化免疫抑制治疗期间出现巨细胞病毒感染和慢性肝炎。随后检测出B型胸腺瘤。尽管进行了手术或放疗以及强化药物治疗，患者分别在7年和1年后死亡。
某些与长期存在的胸腺瘤相关的感染/皮肤表现可能预示着它们的晚期出现，尽管之前进行了胸腺切除术。
