Case Report: Longitudinal Extensive Transverse Myelitis With Novel Autoantibodies Following Two Rounds of Pembrolizumab

Frontiers in Neurology

Anna Christine Nilsson¹  Salma Charabi²  Lotte Engell-Noerregaard³  Christian Stenör⁴ 

[1] Autoimmune Laboratory, Department of Clinical Immunology, Odense University Hospital, Odense, Denmark;Department of Neurology, University of Copenhagen Herlev Hospital, Herlev, Denmark;Department of Oncology, University of Copenhagen Herlev Hospital, Herlev, Denmark;Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark;

关键词: immune checkpoint inhibitors;    transverse myelitis;    immune related adverse events;    pembrolizumab;    case report (source: MeSH NLM);    neurological immune-related adverse effects;   

DOI  :  10.3389/fneur.2021.655283

来源: DOAJ

【 摘 要 】

A 63-year-old male with metastatic non-small cell lung cancer developed longitudinal extensive transverse myelitis (LETM) following two cycles of Pembrolizumab, an immune checkpoint inhibitor (ICI) targeting the programmed cell death receptor 1 (PD-1). Magnetic resonance imaging (MRI) showed centromedullary contrast enhancement at several levels, cerebrospinal fluid (CSF) cytology showed lymphocytic pleocytosis, and indirect immunofluorescence assay (IFA) on the primate cerebellum, pancreas, and intestine revealed strong binding of neuronal autoantibodies to unknown antigens. CSF C–X–C motif ligand 13 (CXCL13) was elevated. The patient was treated with plasma exchange (PEX) and intravenous (i.v.) methylprednisolone (MP) 1 g/day for 5 days followed by oral (p.o.) MP 100 mg/day for 10 days with clinical and radiological response. However, after discontinuation of MP, LETM relapsed and the patient developed paralytic ileus presumably due to autoimmune enteropathy and suffered a fatal gastrointestinal sepsis. Findings of novel neuronal autoantibodies and highly elevated CXCL13 in CSF suggest that the severe neurological immune-related adverse event (nirAE) was B-cell mediated contrary to the commonly assumed ICI-induced T-cell toxicity. An individual evaluation of the underlying pathophysiology behind rare nirAEs is essential for choosing treatment regimens and securing optimal outcome.

【 授权许可】

Unknown