POEMS综合征22例临床分析  

Clinical Analysis of 22 Cases of POEMS Syndrome

作　　者：林洁[1] 朱宏丽[1] 

机构地区：[1]中国人民解放军总医院老年血液科,北京100853

出　　处：《中国实验血液学杂志》2010年第4期1042-1045,共4页Journal of Experimental Hematology

摘　　要：本研究探讨我国POEMS综合征的临床特点。回顾性分析中国人民解放军总医院22例POEMS综合征的临床特征。结果表明:患者平均年龄47.7岁,男∶女=2.67;首发症状以无力最多;神经系统病变见于所有病例,主要表现为无力、麻木,肌电图神经源性损害,脑脊液常有压力和蛋白升高。脑脊液蛋白电泳可见单克隆带阳性;肝脾、淋巴结肿大常见;内分泌病变占95.5%,以性腺功能减退最为常见;M蛋白检查中以免疫固定电泳阳性率最高为92.9%,免疫球蛋白类型中IgA多于IgG,轻链以λ型多见,但小部分患者出现κ型轻链。皮肤改变为色素沉着、多毛、多汗。结论:本组病例临床特征基本上与国内外报道的相似,但与国外报道病例不完全相同,有一定差异。This study was purposed to investigate the clinical characteristics of POEMS syndrome in China, the clinical data of 22 patients with POEMS syndrome admitted in Chinese PLA general hospital from April 1993 to April 2008 were analyzed retrospectively. The restults showed that the average age of patients was 47.7 years old, and the male ： female ratio was 2.67. Weakness was the most frequent initial symptom. Nervous system lesions were found in all cases, weakness and numbness were predominent. Electromyogram showed neurogenic damage. Cerebrospinal fluid pressure and protein level were often elevated. Monoclonal band could be seen in electrophoresis of cerebrospinal fluid. Enlargement of liver, spleen and lymph nodes were common. Endocrine diseases accounted for 95. 5%, in which gonadal dysfunction was most common. The sensitive test in M protein examination was immunofixation electrophoresis with positive rate 92.9%. IgA level was higher than lgG, and h-type light chain was the majority, but K-type light chain appeared in a small number of patients. Changes in skin included hyperpigmentation, hirsutism and sweating. In conclusion, clinical characteristics of patients in this group were basically similar to that reported in home, but were some different from that reported in abroad including following indicators： cerebrospinal fluid protein level, incidence of hepatomegaly and splenomegaly, serum protein level, light chain ~ and h level, incidence of peripheral edema, ascites, pleural effusion and platelet count.

关 键 词：POEMS综合征 周围神经病变 M蛋白 内分泌病变 

分 类 号：R745[医药卫生—神经病学与精神病学] R588[医药卫生—临床医学]