Immunoglobulins to mitigate paraneoplastic Lambert Eaton Myasthenic Syndrome under checkpoint inhibition in Merkel cell carcinoma

被引:16
作者
Dohrn, Maike F. [1 ,2 ,3 ]
Schoene, Ulrike [1 ]
Kueppers, Charlotte [1 ]
Christen, Deborah [4 ]
Schulz, Joerg B. [1 ]
Gess, Burkhard [1 ]
Tauber, Simone [1 ]
机构
[1] Rhein Westfal TH Aachen, Dept Neurol, Med Fac, Aachen, Germany
[2] Univ Miami, Dr John T Macdonald Fdn, Dept Human Genet, Miller Sch Med, Miami, FL 33136 USA
[3] Univ Miami, John P Hussman Inst Human Genom, Miller Sch Med, Miami, FL 33136 USA
[4] Rhein Westfal TH Aachen, Dept Oncol & Hematol, Med Fac, Aachen, Germany
关键词
D O I
10.1186/s42466-020-00099-5
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
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