【摘要】 目的加深临床医师对POEMS综合征的认识,比较国内外POEMS综合征患者的临床特征。方法回顾性分析北京协和医院32例POEMS综合征患者的临床特征,并与国内外大宗病例报告进行比较。结果32例患者,平均年龄45·8岁,男女比例2·2∶1。临床特征(1)多发性周围神经病(P)麻木(84·4%)、感觉异常(87·5%)、肌无力(90·6%)等。肌电图92·9%为神经源性改变,神经活检75·0%为轴索变性。(2)脏器肿大(O)肝肿大71·9%、脾肿大87·5%、淋巴结肿大62·5%。9例淋巴结活检中5例为Castleman病。(3)内分泌病变(E)男性勃起功能障碍72·7%、女性月经改变60·0%。(4)单克隆浆细胞增生(M)血清M蛋白阳性率72·0%(18/25)。(5)皮肤色素沉着(S)93·8%的患者有皮肤色素沉着。与其他报道相比,临床特征基本相似,但视乳头水肿、脾肿大、多汗等方面有一定差异;M蛋白的发生率与国外报道无差异,显著高于国内既往报道。结论本组POEMS综合征临床特征与国外基本相似,血清M蛋白的发生率并不低于国外。免疫电泳以及骨髓活检轻链组化染色可能有助于提高“M”的检测敏感性。更多还原

【Abstract】 Objective To compare the clinical features of POEMS(polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome patients from Peking Union Medical College Hospital(PUMCH) with those of patients in previous reports. Methods Thirty-two patients with POEMS syndrome were identified in PUMCH over the last two decades. The clinical features of these patients were compared with those in other large series from Japan, China, and the United States. Results The average age at presentation was 45.8 years; 22 were men. All 32 patients had polyneuropathy and decreased knee-jerk reflex. Organomegaly was present in 31 patients. Nine patients underwent lymph node biopsy and 5 of them were proven to have Castleman′s disease. Twenty-six patients had at least one endocrine abnormality at time of presentation, not including more commonly prevalent diseases like diabetes mellitus or hypothyroidism. Seventy-two percent (18/25) of the patients had a detectable monoclonal protein with serum immunofixation, but only 16.7% (4/24) had positive results with serum protein electrophoresis. Skin changes were documented in 30 patients. In comparison with other series, we found a higher prevalence of organomegaly and papilloedema. The rates of M protein level, however, were similar. Conclusions Most features of our series are similar to those from previous foreign series. The prevalence of M protein in our study was similar to that in previous foreign series, but was not as low as reported in previous Chinese studies. Immunofixation and immunohistochemical staining of bone marrow biopsies are probably more sensitive in detecting M protein as compared with serum protein electrophoresis.更多还原