Case report: Granzyme-B expression by T- and B- cells during severe AQP4-positive Neuromyelitis Optica spectrum disorder with fatal venous thromboembolism outcome
BackgroundThe 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)...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-08-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2023.1208977/full |
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| author | Vinícius Oliveira Boldrini Vinícius Oliveira Boldrini Mariana Rabelo Brito Raphael Patrício Silva Quintiliano Lucas Scárdua Silva Lucas Scárdua Silva Clarissa Lin Yasuda Clarissa Lin Yasuda Fernando Cendes Fernando Cendes Alessandro Santos Farias Alessandro Santos Farias Alfredo Damasceno Alfredo Damasceno |
| author_facet | Vinícius Oliveira Boldrini Vinícius Oliveira Boldrini Mariana Rabelo Brito Raphael Patrício Silva Quintiliano Lucas Scárdua Silva Lucas Scárdua Silva Clarissa Lin Yasuda Clarissa Lin Yasuda Fernando Cendes Fernando Cendes Alessandro Santos Farias Alessandro Santos Farias Alfredo Damasceno Alfredo Damasceno |
| author_sort | Vinícius Oliveira Boldrini |
| collection | DOAJ |
| description | BackgroundThe 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 presentationHere, 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.ConclusionsFatal VTE may be a rare outcome, particularly in patients exhibiting SM, and may share poorly understood immunological mechanisms with AQP4-positive NMOSD severity. |
| first_indexed | 2024-03-12T14:25:21Z |
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| id | doaj.art-7ece9f9fc1c44cac976d378cef7931fe |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-03-12T14:25:21Z |
| publishDate | 2023-08-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-7ece9f9fc1c44cac976d378cef7931fe2023-08-18T07:38:49ZengFrontiers Media S.A.Frontiers in Neurology1664-22952023-08-011410.3389/fneur.2023.12089771208977Case report: Granzyme-B expression by T- and B- cells during severe AQP4-positive Neuromyelitis Optica spectrum disorder with fatal venous thromboembolism outcomeVinícius Oliveira Boldrini0Vinícius Oliveira Boldrini1Mariana Rabelo Brito2Raphael Patrício Silva Quintiliano3Lucas Scárdua Silva4Lucas Scárdua Silva5Clarissa Lin Yasuda6Clarissa Lin Yasuda7Fernando Cendes8Fernando Cendes9Alessandro Santos Farias10Alessandro Santos Farias11Alfredo Damasceno12Alfredo Damasceno13Neuroimaging Laboratory, Department of Neurology, University of Campinas, Campinas, São Paulo, BrazilBrazilian Institute of Neuroscience and Neurotechnology (BRAINN), University of Campinas, Campinas, São Paulo, BrazilBrazilian Institute of Neuroscience and Neurotechnology (BRAINN), University of Campinas, Campinas, São Paulo, BrazilNeuroimmunology Unit, Department of Genetics, Evolution, Microbiology and Immunology, Institute of Biology, University of Campinas, Campinas, São Paulo, BrazilNeuroimaging Laboratory, Department of Neurology, University of Campinas, Campinas, São Paulo, BrazilBrazilian Institute of Neuroscience and Neurotechnology (BRAINN), University of Campinas, Campinas, São Paulo, BrazilNeuroimaging Laboratory, Department of Neurology, University of Campinas, Campinas, São Paulo, BrazilBrazilian Institute of Neuroscience and Neurotechnology (BRAINN), University of Campinas, Campinas, São Paulo, BrazilNeuroimaging Laboratory, Department of Neurology, University of Campinas, Campinas, São Paulo, BrazilBrazilian Institute of Neuroscience and Neurotechnology (BRAINN), University of Campinas, Campinas, São Paulo, BrazilNeuroimmunology Unit, Department of Genetics, Evolution, Microbiology and Immunology, Institute of Biology, University of Campinas, Campinas, São Paulo, BrazilAutoimmune Research Laboratory, Department of Genetics, Evolution, Microbiology and Immunology, Institute of Biology, University of Campinas, Campinas, São Paulo, BrazilNeuroimaging Laboratory, Department of Neurology, University of Campinas, Campinas, São Paulo, BrazilBrazilian Institute of Neuroscience and Neurotechnology (BRAINN), University of Campinas, Campinas, São Paulo, BrazilBackgroundThe 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 presentationHere, 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.ConclusionsFatal VTE may be a rare outcome, particularly in patients exhibiting SM, and may share poorly understood immunological mechanisms with AQP4-positive NMOSD severity.https://www.frontiersin.org/articles/10.3389/fneur.2023.1208977/fullDevic's syndromedeep vein thrombosis (DVT)pulmonary thromboembolismT lymphocytesB cellsgranzyme-B |
| spellingShingle | Vinícius Oliveira Boldrini Vinícius Oliveira Boldrini Mariana Rabelo Brito Raphael Patrício Silva Quintiliano Lucas Scárdua Silva Lucas Scárdua Silva Clarissa Lin Yasuda Clarissa Lin Yasuda Fernando Cendes Fernando Cendes Alessandro Santos Farias Alessandro Santos Farias Alfredo Damasceno Alfredo Damasceno Case report: Granzyme-B expression by T- and B- cells during severe AQP4-positive Neuromyelitis Optica spectrum disorder with fatal venous thromboembolism outcome Frontiers in Neurology Devic's syndrome deep vein thrombosis (DVT) pulmonary thromboembolism T lymphocytes B cells granzyme-B |
| title | Case report: Granzyme-B expression by T- and B- cells during severe AQP4-positive Neuromyelitis Optica spectrum disorder with fatal venous thromboembolism outcome |
| title_full | Case report: Granzyme-B expression by T- and B- cells during severe AQP4-positive Neuromyelitis Optica spectrum disorder with fatal venous thromboembolism outcome |
| title_fullStr | Case report: Granzyme-B expression by T- and B- cells during severe AQP4-positive Neuromyelitis Optica spectrum disorder with fatal venous thromboembolism outcome |
| title_full_unstemmed | Case report: Granzyme-B expression by T- and B- cells during severe AQP4-positive Neuromyelitis Optica spectrum disorder with fatal venous thromboembolism outcome |
| title_short | Case report: Granzyme-B expression by T- and B- cells during severe AQP4-positive Neuromyelitis Optica spectrum disorder with fatal venous thromboembolism outcome |
| title_sort | case report granzyme b expression by t and b cells during severe aqp4 positive neuromyelitis optica spectrum disorder with fatal venous thromboembolism outcome |
| topic | Devic's syndrome deep vein thrombosis (DVT) pulmonary thromboembolism T lymphocytes B cells granzyme-B |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2023.1208977/full |
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