病例报告：严重的水通道蛋白4阳性视神经脊髓炎谱系障碍伴致命性静脉血栓栓塞结局时T细胞和B细胞中的颗粒酶B表达
Case report: Granzyme-B expression by T- and B- cells during severe AQP4-positive Neuromyelitis Optica spectrum disorder with fatal venous thromboembolism outcome.
作者信息
Boldrini Vinícius Oliveira, Brito Mariana Rabelo, Quintiliano Raphael Patrício Silva, Scárdua Silva Lucas, Yasuda Clarissa Lin, Cendes Fernando, Farias Alessandro Santos, Damasceno Alfredo
机构信息
Neuroimaging Laboratory, Department of Neurology, University of Campinas, Campinas, São Paulo, Brazil.
Brazilian Institute of Neuroscience and Neurotechnology (BRAINN), University of Campinas, Campinas, São Paulo, Brazil.
出版信息
Front Neurol. 2023 Aug 17;14:1208977. doi: 10.3389/fneur.2023.1208977. eCollection 2023.
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 CD8GzmB T, CD4GzmB T lymphocytes, and residual CD19GzmB 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.
背景
循环CD8 T淋巴细胞中丝氨酸蛋白酶颗粒酶B（GzmB）的表达最近被认为是视神经脊髓炎谱系障碍（NMOSD）患者免疫治疗反应不佳和严重残疾的生物标志物。同时，静脉血栓栓塞（VTE）主要在表现为横贯性脊髓炎的NMOSD患者中被报道。
病例介绍
在此，我们描述了一名水通道蛋白4阳性（AQP4阳性）的NMOSD患者，该患者表现为短程脊髓炎（SM），并在抗CD20治疗期间发生致命的肺血栓栓塞/下肢深静脉血栓形成。外周血的流式细胞术分析显示，循环CD8GzmB T、CD4GzmB T淋巴细胞和残余CD19GzmB B细胞的细胞毒性行为增强。
结论
致命性VTE可能是一种罕见的结果，尤其是在表现为SM的患者中，并且可能与AQP4阳性NMOSD的严重程度存在尚未完全了解的免疫机制。
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