Ruck Tobias, Huntemann Niklas, Öztürk Menekse, Schreiber Stefanie, Lichtenberg Stefanie, Masanneck Lars, Nelke Christopher, Ben Moussa Hend, Ulrych Thomas, Seifert Marc, Mougiakakos Dimitrios, Dietrich Sascha, Meuth Sven G
Ruhr University Bochum, BG University Hospital Bergmannsheil, Department of Neurology, 44789 Bochum, Germany; BG University Hospital Bergmannsheil, Heimer Institute for Muscle Research, 44789 Bochum, Germany; Department of Neurology, University Hospital and Medical Faculty Düsseldorf, Heinrich Heine University Düsseldorf, 40225 Düsseldorf, Germany.
Department of Neurology, University Hospital and Medical Faculty Düsseldorf, Heinrich Heine University Düsseldorf, 40225 Düsseldorf, Germany.
Mol Ther. 2025 Sep 3;33(9):4135-4142. doi: 10.1016/j.ymthe.2025.06.042. Epub 2025 Jun 28.
In this case series, we report the first off-label use of the CD19xCD3 T cell engager blinatumomab in two patients with generalized myasthenia gravis (MG). Refractory MG remains a major therapeutic challenge, with patients experiencing severe disability and potentially life-threatening crises despite intensive immunotherapy. This study evaluates the clinical efficacy and safety of short-term blinatumomab treatment in two patients with severe, refractory generalized MG. Both individuals had been experiencing persistent disease burden with myasthenic crises leading to severe disability, despite multimodal immunotherapy. Following treatment with blinatumomab, both patients showed rapid and sustained clinical improvements, reflected in significant reductions in MG-specific scores (MG Activities of Daily Living scale, Quantitative MG score, and revised MG Quality of Life-15), further patient-reported outcomes, digital activity markers, and gait analyses. Laboratory findings revealed persistent B cell depletion in patient 1, whereas patient 2 demonstrated clinical improvement and autoantibody reduction despite B cell repopulation by day 106. Both patients experienced grade 1 cytokine release syndrome during initial treatment phases, but no neurotoxicity or severe adverse events were observed. This report underscores the potential of CD19xCD3 T cell engagers as a promising therapeutic approach in severe autoimmune neuroimmunological disorders, warranting further investigation in clinical trials and mechanistic studies.
在本病例系列中,我们报告了CD19xCD3 T细胞衔接子博纳吐单抗在两名全身型重症肌无力(MG)患者中的首次非标签使用情况。难治性MG仍然是一个重大的治疗挑战,尽管进行了强化免疫治疗,患者仍会出现严重残疾并可能面临危及生命的危机。本研究评估了短期使用博纳吐单抗治疗两名重度难治性全身型MG患者的临床疗效和安全性。尽管接受了多模式免疫治疗,但这两名患者均持续承受疾病负担,出现重症肌无力危象,导致严重残疾。使用博纳吐单抗治疗后,两名患者均迅速且持续地出现临床改善,这体现在MG特异性评分(MG日常生活活动量表、定量MG评分和修订的MG生活质量-15)显著降低、患者报告的进一步结果、数字活动标志物和步态分析中。实验室检查结果显示患者1持续存在B细胞耗竭,而患者2尽管在第106天时B细胞重新增殖,但仍出现临床改善且自身抗体减少。两名患者在初始治疗阶段均经历了1级细胞因子释放综合征,但未观察到神经毒性或严重不良事件。本报告强调了CD19xCD3 T细胞衔接子作为一种有前景的治疗方法在严重自身免疫性神经免疫疾病中的潜力,值得在临床试验和机制研究中进一步探讨。