自生造血干细胞移植治疗系统性硬化症后出现重症肌无力新发病例:持续自身免疫还是耐受重置不足?
New onset of myasthenia gravis after treatment of systemic sclerosis by autologous hematopoietic stem cell transplantation: sustained autoimmunity or inadequate reset of tolerance?
机构信息
Department of Internal Medicine and Rheumatology, La Meynard Hospital, Fort de France, France.
出版信息
Hum Immunol. 2010 Apr;71(4):363-5. doi: 10.1016/j.humimm.2010.01.013. Epub 2010 Feb 4.
Autologous hematopoietic stem-cell transplantation (HSCT) showed promising results for the treatment of primary severe autoimmune diseases (ADs). In this context, development of secondary AD after HSCT has exceptionally been observed, further questioning the roles of patient propensity for AD and of the HSCT procedure. Herein, we report new onset of myasthenia gravis 3 years after successful HSCT in a patient with severe systemic sclerosis, while in complete remission from her first AD. The de novo occurrence of secondary AD (myasthenia gravis) after HSCT was accompanied by the appearance of clonal T-cell expansions measured by the "immunoscope" technique in the context of an ongoing T-cell immune reconstitution. Secondary ADs are increasingly recognized after HSCT for AD. In our case, development of myasthenia followed clonal T-cell expansion. Detailed T-cell repertoire analysis may shed light on autoreactivity mechanisms after HSCT and may help to identify patients at risk.
自体造血干细胞移植(HSCT)在治疗原发性严重自身免疫性疾病(AD)方面显示出良好的效果。在此背景下,HSCT 后发生继发性 AD 极为罕见,这进一步质疑了患者发生 AD 的倾向和 HSCT 程序的作用。本文报告了一例严重系统性硬化症患者在 HSCT 成功 3 年后新发重症肌无力,而患者此前的原发性 AD 已完全缓解。HSCT 后新发生的继发性 AD(重症肌无力)伴“免疫镜”技术检测到的克隆性 T 细胞扩增,此时 T 细胞免疫重建仍在持续。HSCT 后继发性 AD 越来越常见。在我们的病例中,重症肌无力的发生紧随 T 细胞克隆性扩张之后。详细的 T 细胞受体谱分析可能有助于阐明 HSCT 后的自身反应机制,并帮助识别高危患者。