病例报告：胸腺瘤相关重症肌无力中的持续性低丙种球蛋白血症：利妥昔单抗或古德综合征的影响？
Case report: Persistent hypogammaglobulinemia in thymoma-associated myasthenia gravis: the impact of rituximab or Good's syndrome?
作者信息
Ren Jingru, Wang Jianchun, Liu Ran, Guo Jing, Yao Yan, Luo Jingjing, Hao Hongjun, Gao Feng
机构信息
Department of Neurology, Peking University First Hospital, Beijing, China.
出版信息
Front Neurol. 2023 May 5;14:1152992. doi: 10.3389/fneur.2023.1152992. eCollection 2023.
INTRODUCTION
Rituximab (RTX) showed good efficacy and safety for patients with myasthenia gravis. However, the percentage of peripheral CD20+ B cell may be absent for years after low dose of RTX treatment. Persistent hypogammaglobulinemia and opportunistic infection may occur in patients under treatment of RTX with thymoma relapse.
CASE REPRESENTATION
We report a case of refractory myasthenia gravis. After two doses of 100 mg rituximab, the patient developed transient neutropenia. The peripheral blood CD20+ B cell percentage was 0 more than 3 years. Eighteen months later, the patient's symptoms relapsed with thymoma recurred. She had persistent hypogammaglobulinemia and multiple opportunistic infections.
CONCLUSION
In MG patient under B cell depletion therapy had thymoma relapse, Good's syndrome may induce prolonged B cell depletion, hypogammaglobulinemia and opportunistic infections.
引言
利妥昔单抗（RTX）对重症肌无力患者显示出良好的疗效和安全性。然而，低剂量RTX治疗后数年外周血CD20+B细胞百分比可能会消失。接受RTX治疗且胸腺瘤复发的患者可能会出现持续性低丙种球蛋白血症和机会性感染。
病例报告
我们报告一例难治性重症肌无力病例。在给予两剂100mg利妥昔单抗后，患者出现短暂性中性粒细胞减少。外周血CD20+B细胞百分比在3年多的时间里一直为0。18个月后，患者症状复发，胸腺瘤复发。她患有持续性低丙种球蛋白血症和多次机会性感染。
结论
在接受B细胞清除疗法且胸腺瘤复发的重症肌无力患者中，古德综合征可能导致长期B细胞清除、低丙种球蛋白血症和机会性感染。
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