POEMS综合征17例临床分析

POEMS syndrome:A clinical analysis of 17 cases

目的分析POEMS综合征的临床特征,以提高对本病的诊治水平。方法回顾分析17例POEMS综合征的临床表现、实验室检查与治疗方法。结果17例均有多发性神经病,且为最突出的主诉;皮肤色素沉着16例、脾肿大16例、外周水肿15例是最常见的症状;眼底检查10例中有视乳头水肿6例;性腺、甲状腺机能减退是最常见的内分泌病变;蛋白免疫固定电泳可提高M蛋白的检出率;发现骨髓瘤比例明显偏低。13例经皮质类固醇、三苯氧胺、免疫抑制剂治疗后8例症状缓解。结论POEMS综合征临床表现复杂多样,常易误诊;对以多发性神经病及皮肤色素沉着为突出表现者,应高度怀疑POEMS综合征,及时行血、尿蛋白免疫固定电泳等检查以明确诊断。皮质类固醇、免疫抑制剂治疗有效。

Objective： To analyze the clinical characteristics of POEMS syndrome in order to improve the level of diagnosis and treatment for this disease. Methods：Medical records of 17 in-patients with POEMS syndrome were carefully and retrospectively reviewed and its characteristic was summarized. Results： All patients had polyneuropathy as the most chief complaint. The skin hyperpigmentation, splenomegaly and peripheral edema were the most common manifestations： The papilledema occurred in 60% of the patients. The gonadal dysfunction and hypothyroidism were the most common endocrinopathies. The monoclonal protein was less detected than others. The patients with myeloma in the patients were fewer than others. Some patients had a response to immunosuppressive agents. Conclnsions：The clinical features in the patients resemble those in other countries with own characteristics. Misdiagnosis for POEMS syndrome is frequent. The immunosuppressive agents are effective.