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作为主要诊断标准外,还应该包括硬化性骨病变、Castlem an病、视乳头水肿、外周性水肿和浆膜腔积液、皮肤病变、脏器肿大及内分泌病变在内的次要诊断标准。
Objective 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 the cases reported in literature. Results In addition to the typical characteristics of polyneuropathy （ 100% ） , （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. Conclusion To make the diagnosis of POEMS syndrome, both major and minor criteria are 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.
出处
《基础医学与临床》
CSCD
北大核心
2007年第3期337-341,共5页
Basic and Clinical Medicine
