POEMS综合征14例临床病例分析

Clinical Analysis of 14 Cases of POEMS Syndrome

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 characteristics and treatment of POEMS syndrome, and to improve the level of diagnosis. Methods： A total of 14 patients with POEMS syndrome diagnosed in our hospital from 2002 to 2016 were reviewed retrospectively and reviewed the general data, clinical manifestations, auxiliary examination and treatment regimen. Results： The average age is 50.7 years,and the ratio of male to female was 1： 1. All patients had peripheral neurological symptoms, and more than 70% are as the first symptom. 12 patients had water overload, 9 cases had pigmentation and other skin changes. 92% of patients confirmed monoclonal plasma cell proliferation abnormalities which mainly are Ig A-λ type. Organomegaly, osteosclerosis, papilledema were detected in patients with positive rates were77%, 71% and 100%. All patients were treated with glucocorticoids, half of whom were on CD regimen combined with thalidomide. Conclusion： POEMS syndrome is a rare multi- system damage syndrome. For the patients with peripheral neuropathy form onset and multiple organs involved, further screening test should be done to avoid misdiagnosis.