PDF(Pubmed)
Abstract：
Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes (POEMS) syndrome is a rare disorder with multiple presentations and a constellation of symptoms. We present a 62 year-old female who presented to the Emergency Department for acute dyspnea. Chest Xray showed sclerotic lesions in the ribs and thoracic spine. Further imaging studies with computed tomography (CT) and positron emission tomography (PET) scans were suggestive of a benign process. Improvement was seen with supportive management. A few months later, patient developed neurological symptoms with reduced exercise tolerance. Mixed demyelinating and axonal polyneuropathy was diagnosed by electromyography. Further work up with bone marrow biopsy and immunochemistry testing revealed lambda and kappa plasma cell disorder, with elevated vascular endothelial growth factor (VEGF). Patient was diagnosed with POEMS and initiated on chemotherapy. POEMS syndrome is commonly missed due to its rarity and varied clinical presentations. VEGF plays a crucial role in the diagnosis. Management requires a multidisciplinary approach.
摘要：
多发性神经病,器官肿大,内分泌疾病,M-蛋白,皮肤变化（POEMS）综合征是一种罕见的疾病，具有多种表现和一系列症状。我们介绍了一名62岁的女性，她因急性呼吸困难而向急诊科就诊。胸部X光显示肋骨和胸椎硬化病变。计算机断层扫描（CT）和正电子发射断层扫描（PET）扫描的进一步成像研究暗示了良性过程。通过支持性管理看到了改善。几个月后,患者出现神经系统症状，运动耐量降低。肌电图诊断为混合性脱髓鞘和轴索多发性神经病。进一步的骨髓活检和免疫化学检测显示λ和κ浆细胞疾病，血管内皮生长因子（VEGF）升高。患者被诊断为POEMS并开始化疗。POEMS综合征由于其罕见且临床表现多样，通常被错过。VEGF在诊断中起着至关重要的作用。管理需要多学科方法。
