POEMS综合征六例临床特点分析

Clinical analysis on 6 cases with POEMS syndrome

目的：分析 POEMS 综合征的临床特点，提高临床医生对该病的认识。方法收集海军总医院2005-01—2015-05确诊的 POEMS 综合征6例，回顾分析其临床表现、实验室检查、治疗及预后等资料。结果男5例、女1例，发病年龄39-59岁；疾病初期临床表现可仅累及1个或2个系统，随着病情进展，临床表现为多发性周围神经病、皮肤改变及单克隆浆细胞异常增生6例，脏器及淋巴结肿大5例，内分泌异常5例，视乳头水肿4例，浆膜下积液3例，脑脊液蛋白细胞分离6例，血和脑脊液寡克隆区带阳性2例。行腓肠神经活检2例，病理学检查示活动性轴索病变伴髓鞘脱失。行发射型计算机断层（ECT）检查4例，结果显示骨髓增生性改变3例。行 PET-CT 检查2例，均显示硬化性骨病。误诊为慢性炎性脱髓鞘性多发性神经病（CIDP）4例，慢性周围神经病2例。结论 POEMS 综合征常累及多个系统，常见的受累系统为周围神经系统和单克隆浆细胞异常增生的血液系统，早期易误诊为 CIDP。血免疫固定电泳／PET-CT 检查有助于早期发现该病。

Objective To analyze the clinical features of POEMS syndrome in order to improve the recognition of the disease. Methods Clinical features,auxiliary examination,treatment and prognosis of 6 patients with POEMS syndrome admitted to Navy General Hospital during 2005-01—2015-05 were retrospectively analyzed. Results Five male and one female patients were included,aging from 39-53 years old.Early clinical manifestations of the disease might involve only one or two systems.As the disease progressed,all patients had polyneuropathy,skin changes and monoclonal plasma cell proliferative disorder.Five cases had organs and lymph node enlargement.Five cases had endocrine abnormality.Other clinical features were shown as follows：4 cases had papilledema,3 cases had serous effusion,6 cases performed cerebral spinal fluid （CSF）protein cell separa-tion,2 cases were positive for blood and CSF oligoclonal bands.Sural nerve biopsy was performed in 2 cases,the pathological examination showed axonal lesion with myelin depigmentation.Emission computed tomography （ECT）was performed in 4 cases.The results showed 3 cases with myeloproliferative disorders.PET-CT was performed in 2 cases and osteopetrosis was observed in both of them.Four cases were misdiagnosed as chronic in-flammatory demyelinating polyneuropathy （CIDP）,2 cases were misdiagnosed as chronic peripheral neuropathy. Conclusions POEMS syndrome often involves multiple systems,peripheral nervous system and monoclonal plasma cell dysplasia of the blood system are common.POEMS syndrome in the early stage could be easily misdi-agnosed as CIDP,immune serum fixed electrophoresis and PET-CT could contribute to identify the disease.