Case report: Granzyme-B expression by T- and B- cells during severe AQP4-positive Neuromyelitis Optica spectrum disorder with fatal venous thromboembolism outcome

Autor(es)

Boldrini, Vinicius Oliveira ; Brito, Mariana Rabelo ; Quintiliano, Raphael Patricio Silva ; Scardua Silva, Lucas ; Yasuda, Clarissa Lin ; Cendes, Fernando ; Farias, Alessandro Santos ; Damasceno, Alfredo

• Tipo de documento: Artigo Científico
• Fonte: FRONTIERS IN NEUROLOGY; v. 14, p. 6-pg., 2023-08-17.

Resumo

Background The expression of serine protease granzyme-B (GzmB) by circulating CD8(+) T lymphocytes has been recently suggested as a biomarker for poor immunotherapy response and severe disability in patients with Neuromyelitis Optica spectrum disorders (NMOSD). In parallel, venous thromboembolism (VTE) has been reported mainly in NMOSD patients exhibiting transverse myelitis.Case presentation Here, we describe an Aquaporin-4 positive (AQP4-positive) NMOSD patient who showed short myelitis (SM) and experienced a fatal pulmonary thromboembolism/lower extremity deep vein thrombosis during anti-CD20 treatment. Flow cytometry analyses from the peripheral blood revealed an enhanced cytotoxic behavior through circulating CD8(+)GzmB(+) T, CD4(+)GzmB(+) T lymphocytes, and residual CD19(+)GzmB(+) B cells.Conclusions Fatal VTE may be a rare outcome, particularly in patients exhibiting SM, and may share poorly understood immunological mechanisms with AQP4-positive NMOSD severity. (AU)

• Processo FAPESP: 17/21363-5 - Regulação da via JAK/STAT/SOCS na mudança de perfil de Th17 para Th1 (produtoras de IFNg) durante a evolução clínica da encefalomielite experimental autoimune
• Beneficiário: Alessandro dos Santos Farias
• Modalidade de apoio: Auxílio à Pesquisa - Regular
• Processo FAPESP: 13/07559-3 - Instituto Brasileiro de Neurociência e Neurotecnologia - BRAINN
• Beneficiário: Fernando Cendes
• Modalidade de apoio: Auxílio à Pesquisa - Centros de Pesquisa, Inovação e Difusão - CEPIDs