背景:免疫检查点抑制剂(ICIs)可引起一系列不良事件,称为免疫相关不良事件(irAE),类似于自身免疫反应。免疫介导的重症肌无力(MG)是一种罕见且严重的神经系统不良事件,与需要及时治疗的ICIs有关。在耶和华见证人的人群中,这些不良事件的典型管理可能不是选择,和替代治疗的选择将是必要的。
方法:73岁的耶和华见证人患者患有高级未分化多形性肉瘤,在开始pembrolizumab治疗后约4周出现免疫介导的MG。入学那天,患者有3天的下垂恶化史,右大于左。后来发现他对MG具有血清阴性。
方法:患者需要接受吡啶斯的明治疗,类固醇,并同意在出院前进行血浆置换(PLEX)。他的神经症状几乎消失了,和派博利珠单抗停药.他后来接受了左大腿软组织肉瘤的根治性切除术和腹股沟浅表淋巴结清扫术。他现在正在积极监视。
结论:虽然神经系统不良事件通常在ICIs开始后6周出现,据报道,MG最早发生在开始后4周。这种罕见和严重的不良事件需要及时治疗,临床医生需要了解这一独特患者群体的替代治疗方案.关于血液制品和派系的早期对话必须根据患者的个人决定制定治疗计划。
BACKGROUND: Immune checkpoint inhibitors (ICIs) can cause a spectrum of adverse events known as immune-related adverse events (irAEs) that resemble autoimmune responses. Immune-mediated myasthenia gravis (MG) is a rare and serious neurologic adverse event that has been associated with ICIs requiring prompt treatment. In the Jehovah\'s Witness population, typical management of these adverse events may not be options, and alternative treatment choices would be needed.
METHODS: 73-year-old Jehovah\'s Witness patient with high-grade undifferentiated pleiomorphic sarcoma who developed immune-mediated MG approximately 4 weeks after initiation of pembrolizumab. On the day of admission, the patient presented with a three-day history of worsening ptosis, right greater than left. He was later found to be seronegative for MG.
METHODS: The patient required therapy with pyridostigmine, steroids, and agreed to plasma exchange (PLEX) prior to discharge. He achieved near resolution of his neurologic symptoms, and pembrolizumab was discontinued. He later underwent radical resection of the left thigh soft tissue sarcoma and superficial inguinal lymph node dissection. He is now on active surveillance.
CONCLUSIONS: While neurologic adverse events typically present 6 weeks after initiation of ICIs, MG has been reported occurring as early as 4 weeks after initiation. This rare and serious adverse event requires prompt treatment, and clinicians need to be aware of the alternative treatment options in this unique patient population. Early conversations regarding blood products and factions must be had to develop a treatment plan in accordance with the patient\'s personal decisions.