20例POEMS综合征临床病例分析_董漪－副本.pdfVIP

中国临床神经科学2009 年第 17 卷第5 期 494 9, 17 (5) ,494~498 论 著 20 例POEM S 综合征临床病例分析 董 漪，董 强，丁宏岩 (复旦大学附属华山医院神经内科200040) 关键词 POE M S 综合征；回顾性研究；多发性周围神经病 摘要 目的：分析POEM S 综合征的临床表现、治疗与预后的关系。方法：采用回顾性分析、整群抽取的研究方法对 复旦大学附属华山医院2002 年1 月至2008 年11 月出院的POEMS 综合征患者病史资料进行统计分析。结果：POEMS 综合征多见于50~60 岁男性，以周围神经症状为首发，实验室检查多可发现脑脊液中蛋白升高及骨扫描异常。以激素、 免疫球蛋白及免疫抑制剂治疗。结论：P OEM S 综合征系全身性疾病，可予免疫调节或抑制剂治疗。 Analysis for Clinical Features on 20 Cases of POEMS Syndrome D ONG Yi, D ON G Qiang, DING Hong- Yan Department of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, China KEY WORDS POEMS syndromes; retrospective study; polyneuropathy AB STRACT Aim: To describe clinical featur es of patients with POEMS syndrome and analyze the factors associated with survival. Methods: From Jan. 2002 to Nov. 2008, 20 POEMS syndrome patients were admitted in Huashan Hospital, Fudan University . Records of the cases were statistically analyzed. Results: The onset of POEMS syndrome occurs most frequently in 50~60 year old male patients. The polyneuropathy is associated with POEMS syndrome at first. Elevated level of protein in cerebral spinal fluid and abnormal in bone scan could b e associated with in most cases. Patients wer e tr eated with immunor egulant, such as glucocorticosteroid and gamma globulin, or immunodepressant. Conclusion: POEMS syndrome is a multisystemic devastating syndrome, and may be treated with immunoregulant or immunodepr essant . POEMS综合征(POEMS syndromes)又称Crow Fukase 对象与方法 综合征，是以多系统受累为临床特点，主要包括多发性 神经病(polyneuropathy) 、脏器肿大(organomegaly) 、内分 一般资料 收集复旦大学附属华山医院2002 年1 月 泌病变(endocrinopathy) 、M- 蛋白(M-protein) 、皮肤改变 1 日至20