POEMS综合征临床特征及预后

目的: 研究POEMS综合征的临床特征和预后.方法: 回顾性的分析并随访了我院近10年确诊的21例POEMS综合征患者,并与国外两组大型研究进行比较.结果: 主要临床特征为:多发性周围神经病、皮肤改变、内分泌疾病、水肿渗出、脏器肿大、视乳头水肿、骨损害、贫血及慢性腹泻.与国外研究不同的是:血小板增多症和红细胞增多症频率较低.随访14人,8例死亡,生存中值50.2月.结论: 除传统的5个临床特征外,骨骼损害、水肿、和视乳头水肿等亦应为该病的临床诊断特征.慢性腹泻及贫血可能是本病较多的伴发症状.本病常见的死亡原因是:肾脏、肺脏和心脏功能衰竭.

The Clinical Features and Prognosis of POEMS Syndrome

Objective:To analyze the clinical features and prognosis of POEMS syndrome.Methods:Retrospective analysis of 21de nova cases POEMS syndrome diagnosed in West China Hospital in last 10 years and comparing the findings in this study with those of the other two largest patient series.Results:The major clinic features are shown as following:polyneuropathy,sikn change,endocrinopathy,edema and effusions,organomegaly,papilloedema,bone lesion,anemia and chronic diarrhea,but lower frequency of thrombocytosis and erythrocytosis is different from what is reported by foreign studies.Of the 14 cases who we managed to follow up,8 have been dead.The median survival time is 50.2 months.Conclusion:Besides the classic pentads,bone lesion,edema and papilloedema should be included into the diagnostic criteria.Chronic diarrhea and anemia are probably the complications.Common causes of death are renal,respiratory or/and cardiac failure.