POE MS综合征13例临床分析

Clinical analysis on 13 cases of POEMS syndrome

目的 分析研究POEMS综合征的临床特征、以提高对本症的诊治水平。方法 采用回顾性分析方法对 13例POEMS综合征进行分析。结果 13例患者中 ,4例伴骨髓瘤 ,其他最主要临床表现为多发性神经病变 (10 0 % ) ,皮肤色素沉着 (84.6 % ) ,水肿 (84.6 % ) ,肝、脾肿大 (6 9.0 %及6 1.5 % )及内分泌病变 (84.6 % )。院外 13例均误诊 ;入院后误诊 9例 ;11例经治疗后症状缓解 ,2例自动出院。结论 POEMS综合征仍是一个被人们认识不足的疾病 ,因此应予以重视。

Objective To analyse the clinical characteristics of POEMS syndrome and to improve the diagnosis and treatment for POEMS syndrome. Methods Thirteenmedicalrecordsofin patientswith POEMS syndrome were carefully and retrospectively reviewed. Results Of 13 cases, 4 had myeloma. The majorclinicalmanifestationsofthesepatientswereperipheralpolyneuropathy(100%), pigmentation (84.6%),edema (84.6%),hepato andspleno megaly(69.0%and61.5%)andendocrinopathy (84.6%). The misdiagnosis rate was 100% before admission and 70% after admission. Symptoms were improved in 11 cases after treatment with prednisone or immuno suppressive agents. Conclusions POEMS syndrome, at present, remains to be an underdiagnosed and undertreated disorder and more attention should be paid to it for improving the accuracy of diagnosis and adequacy of treatment.