Dohrn Maike F, Schöne Ulrike, Küppers Charlotte, Christen Deborah, Schulz Jörg B, Gess Burkhard, Tauber Simone
Department of Neurology, Medical Faculty of the RWTH Aachen University, Aachen, Germany.
Dr. John T. Macdonald Foundation, Department of Human Genetics and John P. Hussman Institute for Human Genomics, University of Miami, Miller School of Medicine, Miami, Florida USA.
Neurol Res Pract. 2020 Dec 9;2:52. doi: 10.1186/s42466-020-00099-5. eCollection 2020.
Lambert-Eaton myasthenic syndrome (LEMS) is a rare, autoimmune or paraneoplastic condition characterized by muscle weakness and fatigability. In cancer therapy, immune checkpoint inhibitors (ICI) sensitize the immune system for tumor antigens. We report a 62-year-old, female patient with paraneoplastic LEMS as first manifestation of Merkel cell carcinoma. Under avelumab, the LEMS exacerbated with worsening of limb weakness and a severely reduced vital capacity (< 1 l). To treat this immunological side effect, we added a regimen with intravenous immunoglobulins. Hereby, the LEMS improved significantly. As we were able to continue the cancer treatment, the Merkel cell carcinoma has been in remission so far. This is the first description of paraneoplastic LEMS, avelumab, and Merkel cell carcinoma. We conclude that immunoglobulins are an option to control an ICI-associated deterioration of paraneoplastic symptoms.
兰伯特-伊顿肌无力综合征(LEMS)是一种罕见的自身免疫性或副肿瘤性疾病,其特征为肌肉无力和易疲劳。在癌症治疗中,免疫检查点抑制剂(ICI)可使免疫系统对肿瘤抗原敏感。我们报告了一名62岁的女性患者,其副肿瘤性LEMS是默克尔细胞癌的首发表现。在使用阿维鲁单抗治疗期间,LEMS病情加重,肢体无力恶化,肺活量严重降低(<1升)。为治疗这种免疫副作用,我们添加了静脉注射免疫球蛋白方案。由此,LEMS明显改善。由于我们能够继续进行癌症治疗,默克尔细胞癌至今一直处于缓解状态。这是关于副肿瘤性LEMS、阿维鲁单抗和默克尔细胞癌的首次描述。我们得出结论,免疫球蛋白是控制ICI相关副肿瘤症状恶化的一种选择。