POEMS综合征的诊断和治疗  

Diagnosis and treatment of POEMS syndrome

作　　者：管宇宙[1] 牛婧雯[1] Guan Yuzhou;Niu Jingwen(Department of Neurology,Beijing Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院神经科,北京100730

出　　处：《中华神经科杂志》2023年第4期448-452,共5页Chinese Journal of Neurology

摘　　要：POEMS综合征是罕见的浆细胞异常增殖导致的副肿瘤综合征,主要表现为脱髓鞘性周围神经病及多系统受累。其诊断的主要标准包括多发性周围神经病、克隆性浆细胞增殖、硬化性骨病变、血管内皮生长因子升高、卡斯尔曼病;次要标准包括脏器肿大、内分泌异常、特征性皮肤改变、视乳头水肿、血管外容积增加、血小板增多。详细的病史、体检及必要的辅助检查包括骨X射线体层摄影检查、血管内皮生长因子测量、骨髓活组织检查、肌电图等,可协助该综合征与慢性炎性脱髓鞘性周围神经病以及其他浆细胞疾病(包括淀粉样变性、骨髓瘤、意义未明的单克隆免疫球蛋白病周围神经病)相鉴别。早期诊断和治疗可明显改善疾病的预后。POEMS syndrome is a rare paraneoplastic syndrome due to plasma cell neoplasm.It is a disorder involving multiple organs,mainly manifesting as demyelinating peripheral neuropathy.The major diagnostic criteria for the syndrome are polyradiculoneuropathy,clonal plasma cell disorder,sclerotic bone lesions,elevated vascular endothelial growth factor,and the presence of Castleman disease.Minor features include organomegaly,endocrinopathy,characteristic skin changes,papilledema,extravascular volume overload,and thrombocytosis.A good history and physical examination followed by appropriate testing-radiographic assessment of bones,measurement of vascular endothelial growth factor,bone marrow biopsy and electromyography can differentiate this syndrome from other conditions such as chronic inflammatory demyelinating polyradiculoneuropathy,other plasma disorders(including amyloidosis,myeloma,and monoclonal gammopathy of undetermined significance neuropathy).Early diagnosis and treatment could greatly improve the prognosis.

关 键 词：POEMS综合征 诊断 治疗 

分 类 号：R733[医药卫生—肿瘤]