Margaret Upchurch1, Nicole Caunt1, Jessica McDermott2, Jeffrey Bennett1, Amanda Piquet1
1Neurology, 2Medical Oncology, University of Colorado
Objective:
To report a case of a 66-year-old woman with a history of aquaporin-4-IgG seropositive (AQP4-IgG+) Neuromyelitis Optica Spectrum Disorder (NMOSD) and concurrent metastatic oral cavity squamous cell carcinoma (SCC) who presented with acute left-sided hemiplegia in the setting of pembrolizumab therapy.
Background:
Pembrolizumab is an immune checkpoint inhibitor (ICI) approved for the treatment of several malignancies. ICIs are known to cause a wide variety of immune-related adverse events. While there have been no studies on the safety of ICIs in NMOSD patients, there was a case reported of newly-diagnosed NMOSD following nivolumab treatment. This is a novel case of an NMOSD attack following pembrolizumab treatment in an AQP4-IgG+ NMOSD patient on long-term anti-CD19 B cell depleting therapy.
Design/Methods:
Case report
Results:
A 66-year-old woman with AQP4-IgG+ NMOSD, diagnosed in 2015 and treated with inebilizumab, was diagnosed with metastatic SCC of the tongue in February 2022. She had received inebilizumab every six months from February 2016 to October 2021; infusion was cancelled in April 2022 due to cytotoxic chemotherapy (CD19+ 2 cells/ml; CD27+ 0 cells/ml). Despite resective surgery of the tongue and adjuvant concurrent chemotherapy and radiation, she was found to have widely metastatic SCC in early August 2022 (CD19+ 29 cells/ml; CD27+ 17 cells/ml; CD38+IgM- 12 cells/ml). She was administered inebilizumab and then started on pembrolizumab twelve days later. Ten days after receiving her first pembrolizumab therapy, the patient developed acute onset left-sided weakness (CD19+ 4 cells/ml; CD27+ 0 cells/ml; CD38+IgM- 0 cells/ml). She was found to have a large, active cervical spine lesion consistent with a new NMOSD attack; serum AQP4-IgG had increased from 1:10,000 to 1:100,000.
Conclusions:
An NMOSD attack occurred rapidly following pembrolizumab in a B-cell depleted patient, suggesting a role for T cell activation in relapse. ICIs should be used with caution in NMOSD patients.