以水肿为首发症状的POEMS综合征1例报告

One Case Report of POEMS Syndrome with Edema as the Initial Manifestation

目的:了解POEMS综合征临床特点及诊疗思路;方法:对1例以水肿为临床表现的POEMS综合征进行临床分析及近几年临床文献复习;结果:患者存在双下肢凹陷性水肿,慢性进展性感觉运动性多发性神经表现,伴有色素沉着、多毛、白甲等皮肤改变,辅助检查提示肝脾等器官肿大,甲状腺功能减退,硬化性骨改变,Ig A、λ泳道发现异常单克隆免疫球蛋白,最终诊断为POEMS综合征;结论:POEMS综合征误诊率极高,且预后差,治疗方案仍在进一步研究中,早期的诊断对提高患者的生存率及改善患者的生活质量是及其重要的。

Objective： To summarize the clinical features and diagnosis and treatment of POEMS syndrome. Methods：Clinical analysis of 1 cases of POEMS syndrome with edema as a clinical manifestation and review of clinical literature in recent years.Results The patient presented with sunken edema of both lower extremities and chronic progressive sensorimotor multiple nerve manifestations, Accompanied by pigmentation, hirsutism, white nails and other skin changes,combined assisted examination diagnosed POEMS syndrome. Conclusion： The misdiagnosis rate of POEMS syndrome is very high, early diagnosis is important for improving patient survival and improving patient quality of life.