目的:总结POEMS综合征的临床特征及治疗措施,提高对其诊断、治疗的认识。方法:对2002-2016年在本院诊断POEMS综合征的14例患者的病历资料进行回顾性分析,并复习相关文献。结果:14例平均年龄50.7岁,所有患者均有周围神经症状,并且超过七成以此为首发症状,12例患者有水负荷过多,9例有色素沉着等皮肤改变,92%的患者通过多种检测方式证实有单克隆浆细胞增殖异常,主要是Ig A-λ型。器官肿大、硬化性骨病、视乳头水肿在被检测的患者中的阳性率分别为77%、71%和100%。所有患者的治疗均使用了激素,其中一半为CD方案联合沙利度胺。结论:POEMS综合征是一种较少见的多系统损害综合征,对于以周围神经病变形式起病,伴有全身多系统脏器受累的病因不明患者,应该进行进一步筛查,以免误诊漏诊。 Objective: To summarize the clinical features and treatment measures of POEMS syndrome and to raise awareness of its diagnosis and treatment. Methods: The clinical data of 14 patients with POEMS syndrome diagnosed in our hospital from 2002 to 2016 were analyzed retrospectively and the related literatures were reviewed. Results: The average age of 14 patients was 50.7 years old. All patients had peripheral neurological symptoms, and over 70% were the first symptom. Twelve patients had excessive water load, 9 had skin changes such as pigmentation, and 92% had passed A variety of detection methods confirmed abnormal monoclonal plasma cell proliferation, mainly Ig A-λ type. The positive rates of organ enlargement, sclerosing bone disease and papilledema in patients tested were 77%, 71% and 100%, respectively. All patients were treated with hormones, half of which was CD regimen combined with thalidomide. CONCLUSIONS: POEMS syndrome is a rare multi-system lesion syndrome and should be further screened to avoid misdiagnosis and missed diagnosis for patients with unknown etiology of onset of peripheral neuropathy with systemic multiple organ involvement.