【摘要】 目的:目的:分析POEMS综合征患者的临床特征。方法:回顾性分析中国医科大学附属第一医院20例POEMS综合征患者的临床表现、实验室检查,并复习有关文献。结果:20例POEMS患者中位年龄56岁,男女比例4∶1。临床特点:多发性周围神经病(P)100%、脏器肿大(O)85%、内分泌病变(E)80%、单克隆浆细胞增生(M)100%、皮肤色素沉着(S)100%、水肿及浆膜腔积液70%。其他常见的症状有:血小板增多、贫血、骨骼损害等。结论:POEMS综合征除传统的5个临床特征外,水肿及浆膜腔积液为该病常见的临床特征。免疫固定电泳和(或)M蛋白阴性不能排除本病诊断,反复骨髓穿刺活检以及可疑病变组织活检行免疫组化检查查找单克隆浆细胞证据有利于M-蛋白阴性患者的诊断。更多还原

【Abstract】 Objective: To analyze the clinical manifestation of POEMS syndrome. Methods: Medical records of 20 patients with POEMS syndrome were reviewed and relative literatures were reviewed. Results: Of the 20 patients,the median age was 56 years old,the ratio of male and female was 4∶ 1. The main clinic manifestations were polyneuropathy( P) 100%,organomegaly( O) 100%,endocrinal abnormalities( E) 100%,monoclonal plasma cell proliferation( M) 100%,skin pigmentation( S) 100%,edema and serous effusions 70%. The other common manifestations included thrombocytosis,anemia and bone damage. Conclusion: POEMS syndrome in addition to the traditional five clinical features,edema and serous effusions of the disease is common clinical manifestations,immunofixation electrophoresis and / or M protein negative cannot rule out the diseases,repeated bone marrow biopsy and biopsy with immunohistochemical test of suspicious lesions are useful to find the evidence of monoclonal plasma cells conducive to the diagnosis of the M- protein negative patients.更多还原