POEMS综合征425例临床分析

Clinical Analysis on 425 Cases of POEMS Syndrome

目的总结425例POEMS综合征的临床特点及诊治.方法荟萃总结分析POEMS综合征临床表现的特点并与国外文献相比较.结果 POEMS综合征多见于中年男性,发病年龄11～74岁,平均43岁;临床表现以多发性周围神经病、脏器肿大、内分泌病、M-蛋白和皮肤改变为特征,常与多发性骨髓瘤并存;死因多见于严重感染、呼吸衰竭或心力衰竭等,发病机制不详;激素疗法短期疗效尚可.结论国内POEMS综合征患者周围神经损害、皮肤改变、肢端水肿等较常见,其次为肝脾肿大、阳痿、男性女性化乳房、闭经、胸腹水等.

Objective To summarize the clinical features of reported 425 domestic cases of POEMS syndrome, and its diagnosis and treatment. Methods Clinical characteristics of 425 POEMS cases were summarized with meta analysis. Results POEMS syndrome usually occurred in middle-age males, and its onset age ranged from 11 to 74 years, averaged 43 years. Clinical display was charac- terized by a combination of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. It usually occurred with osteomyeloma. Most patients died of serious infections, respiratory insufficiency or heart failure. The treatment with glycocorticosteroid had led to short-time improvement. Conclusion The patients in our country more present as peripheralneuropathy, skin change and edema, whereas hepatomegaly, splenomegaly, erectile dysfunction, breast enlargement of males, amenorrheal, pleural effusion and ascites are next.