基础医学与临床 ›› 2007, Vol. 27 ›› Issue (3): 337-341.

• 临床园地 • 上一篇    下一篇

POEMS综合征临床表现及诊断:36例病例分析

朱铁楠 华宝来 赵永强   

  1. 北京协和医院血液内科 沈悌
  • 收稿日期:2006-02-22 修回日期:2006-08-28 出版日期:2007-03-25 发布日期:2007-03-25
  • 通讯作者: 朱铁楠

Clinical manifestions and fiangnosis of POEMS syndrime:clinical analusis on 36 cases

  

  • Received:2006-02-22 Revised:2006-08-28 Online:2007-03-25 Published:2007-03-25

摘要: 目的 分析POEMS综合征的临床表现及诊断标准。方法 回顾性分析36例POEMS综合征患者的临床特点,并与国内、外文献进行比较。结果 POEMS综合征患者除了具有多发性神经病(100%)、器官肿大(92%)、内分泌病(86%)、单克隆浆细胞增生(100%)和皮肤病变(86%)的典型临床特征外,还常常表现为血管外容量过负荷(97%)、视乳头水肿(57%)和骨病变(25%)。另外,POEMS综合征患者还常常伴发有Castleman病(25%)。结论 POEMS综合征的诊断除应将多发性神经病和克隆性浆细胞疾病做为主要诊断标准外,还应该包括硬化性骨病变、Castleman病、视乳头水肿、外周性水肿和浆膜腔积液、皮肤病变、脏器肿大、内分泌病变在内的次要诊断标准。

Abstract: Object:To analyze the clinical manifestations and the criteria for the diagnosis of POEMS syndrome. Methods: The clinical characteristics of 36 cases of POEMS syndrome were retrospectively reviewed and compared with literature. Results: Besides of the typical characteristics of polyneuropathy (100%), organomegaly (92%), endocrinopathy (86%), monoclonal plasmaproliferative disorder (100%) and skin changes (86%), the patients of POEMS syndrome also have other important features, including extravascular volume overload (97%), papilledema (57%) and bone lesions (25%). Furthermore, 25% of POEMS syndrome patients have co-existent Castleman disease. Conclusions: To make the diagnosis of POEMS syndrome, major and minor criteria should be required. The former includes polyneuropathy and monoclonal plasmaproliferative disorder and the latter includes osteosclerotic bone lesions, Castleman disease, papilledema, organomegaly, edema or serous cavity effusion, endocrinopathy and skin changes.