POEMS综合征3例报告并文献复习

Cases report and literature review of POEMS syndrome

目的对POEMS综合征的临床特点进行总结,以提高对此病的掌握与鉴别诊断能力。方法报道3例POEMS综合征患者临床表现、实验室检查、治疗方法及转归并复习相关文献。结果 3例患者均存在周围神经病变,其中2例男性患者以双足底踩棉花感为首发表现,逐渐出现双下肢麻木无力、肌肉萎缩、局部水肿、皮肤颜色变黑、男性乳腺发育等症状,1例女性患者以面部、四肢皮肤及乳头变黑为首发表现,逐渐出现足底踩沙感,双下肢麻木无力、闭经、水肿等症状。3例患者免疫固定电泳均检测出M蛋白;1例患者病程中曾查M蛋白阴性,至病程终末期出现血M蛋白及本周氏蛋白尿。3例从首诊到确诊时间分别为16个月、3 y和5 y。对3例患者进行随访观察,1例患者症状改善明显,2例确诊较晚患者死亡。结论 POEMS综合征是一组较少见的与浆细胞病变相关的副肿瘤综合征。临床表现多样,确诊时间较长,早期病例易被误诊,需仔细分析临床及实验室检查资料避免误诊误治。

Objective To analyze the clinical features of POEMS syndrome for an insight into this syndrome. Methods The clinical manifestation,laboratory examination,treatment and outcome of 3 cases with POEMS syndrome were reviewed. Results All cases had peripheral polyneuropathy. 2 male patients began with foot paresthesia as initial symptoms,and then presented with progressive numbness and weakness of lower limbs, muscle atrophy, edema, hyperpigmentation,gynaecomastia. The female patient began with skin pigmentation as first symptoms,then presented with progressive foot paresthesia,numbness and weakness of lower limbs, amenorrhoea, edema. All cases had M-proteins. Durations from initial visit to final diagnosis were 16 months,3 years and 5 years respectively. The clinical symptoms of one patient improved, and other 2 cases died because of delay in diagnosis. Conclusion POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Various clinical manifestations often requires a rather long time to diagnose,and easy to be misdiagnosed in early stage. Careful analysis of clinical and laboratory, data could improve diagnosis accuracy.