Fein Alexander S, Trejo Bittar Humberto E, Shende Manisha R, Scalzetti Ernest M, Ko Melissa W
SUNY Upstate Medical University, Syracuse, NY, USA.
Department of Pathology.
Neuroophthalmology. 2018 Jun 26;43(3):185-191. doi: 10.1080/01658107.2018.1484932. eCollection 2019 Jun.
Castleman disease (CD) is a rare lymphoproliferative disorder that may present with various autoimmune, inflammatory, or neurologic syndromes. This is a case of a 21-year-old woman who presented with signs and symptoms of pseudotumour cerebri (PTC) who subsequently developed myasthenia gravis (MG), and was incidentally found to have a large mass in the posterior mediastinum. Upon resection, the mass was classified as unicentric CD involved with follicular dendritic cell sarcoma. Following treatment with IVIG in the setting of progressive weakness and dyspnea, she has had complete symptom resolution while maintained on a low dose of pyridostigmine for the last two years. There are 13 cases of MG and five cases of optic disc edema described as PTC associated with CD in the literature, but to our knowledge, this is the sole case reported of the intersection of all three conditions in one patient. Increased serum levels of interleukin-6 and vascular endothelial growth factor may provide clues as to the association of CD with these neurologic syndromes.
卡斯特曼病(CD)是一种罕见的淋巴增生性疾病,可能伴有各种自身免疫性、炎症性或神经综合征。这是一名21岁女性的病例,她最初表现为假性脑瘤(PTC)的症状和体征,随后发展为重症肌无力(MG),并偶然发现后纵隔有一个大肿块。切除后,该肿块被分类为伴有滤泡性树突状细胞肉瘤的单中心CD。在因进行性肌无力和呼吸困难接受静脉注射免疫球蛋白治疗后,她的症状已完全缓解,并且在过去两年中一直维持低剂量吡啶斯的明治疗。文献中有13例MG和5例被描述为与CD相关的PTC的视神经盘水肿病例,但据我们所知,这是唯一一例在一名患者中同时出现这三种情况的报告。血清白细胞介素-6和血管内皮生长因子水平升高可能为CD与这些神经综合征的关联提供线索。