PDF(Pubmed)
Abstract：
POEMS syndrome-characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes-is an uncommon and complex paraneoplastic disorder encompassing a diverse array of symptoms. Here we report the challenging case of a 34-year-old female who sought medical attention at the emergency department due to distal lower limb weakness. She was breastfeeding her first child at that time. Her condition rapidly deteriorated, making it difficult for her to perform simple tasks independently. Initially, she struggled with activities like jumping or climbing stairs. Eventually, her ability to walk was also compromised. These symptoms underscored the swift evolution of her polyneuropathy. Nerve conduction studies and electromyography confirmed a diagnosis of mixed demyelinating and axonal polyneuropathy. Subsequent investigations, including bone marrow biopsy and immunochemistry testing, revealed a plasma cell disorder characterized by lambda monoclonal gammopathy, along with elevated levels of vascular endothelial growth factor (VEGF > 8000 pg/mL). This pivotal finding led to the diagnosis of POEMS syndrome, prompting the initiation of antineoplastic therapy (daratumumab-lenalidomide-dexamethasone) to manage this condition. An autologous cell transplantation was planned. The rarity of POEMS syndrome and its diverse clinical manifestations often lead to an incorrect or delayed diagnosis. Our case underscores the importance of considering this syndrome in patients presenting with acute or subacute polyneuropathy, even if the patients are young. In conclusion, this case elucidates the diagnostic complexities of POEMS syndrome, emphasizing the integral role of comprehensive multidisciplinary evaluations and the potential influence of increased VEGF as a diagnostic key element and possible therapeutic target.
摘要：
POEMS综合征-以多发性神经病为特征，器官肿大,内分泌病,M-蛋白,和皮肤变化-是一种罕见且复杂的副肿瘤疾病，包括多种症状。在这里，我们报告了一名34岁女性因下肢远端无力而在急诊科寻求医疗护理的挑战性病例。当时她正在母乳喂养她的第一个孩子。她的病情迅速恶化，使她难以独立完成简单的任务。最初，她在跳跃或爬楼梯等活动中挣扎。最终,她的行走能力也受到了损害。这些症状突显了她的多发性神经病的迅速发展。神经传导研究和肌电图证实了混合脱髓鞘和轴索多发性神经病的诊断。随后的调查，包括骨髓活检和免疫化学检测,揭示了一种以λ单克隆丙种球蛋白病为特征的浆细胞疾病，血管内皮生长因子水平升高(VEGF>8000pg/mL)。这一关键发现导致了POEMS综合征的诊断，提示开始抗肿瘤治疗（达拉图单抗-来那度胺-地塞米松）以控制这种情况。计划进行自体细胞移植。POEMS综合征的稀有性及其多样化的临床表现往往导致诊断错误或延迟。我们的病例强调了在急性或亚急性多发性神经病患者中考虑该综合征的重要性，即使病人很年轻.总之,这个病例阐明了POEMS综合征的诊断复杂性,强调综合多学科评估的整体作用，以及增加VEGF作为诊断关键要素和可能的治疗靶点的潜在影响。
