POEMS综合征5例临床分析

Clinical Analysis of 5 Cases of POEMS Syndrome

背景:POEMS综合征是一组以多发性周围神经病为突出表现的临床症候群。该病少见且临床表现复杂、无特异性,极易漏诊或误诊。目的:总结POEMS综合征的临床特点,提高对该病的认识。方法:回顾性分析上海仁济医院2001年5月～2007年5月5例确诊的POEMS综合征患者的临床表现、实验室检查结果、治疗方案和预后情况。结果:5例POEMS综合征患者的主要临床表现发生率分别为周围神经病变、内分泌异常和皮肤改变100%,脏器肿大80%,M蛋白阳性60%。经激素或免疫抑制剂治疗,出院时4例患者好转,1例恶化,但至随访结束时仅1例维持好转。结论:POEMS综合征临床表现复杂。随着其诊断和治疗的进展,患者预后将明显改善。

Background： POEMS syndrome is a group of disorders with multisystem symptoms characterized by peripheral polyneuropathy. As the syndrome is rare and its clinical manifestations are complicated and nonspecific, it is easily misdiagnosed. Aims： To summarize the clinical characteristics of POEMS syndrome and to improve the recognition of this syndrome. Methods： Five cases of POEMS syndrome diagnosed in Shanghai Renji Hospital from May 2001 to May 2007 were analyzed retrospectively, including their clinical manifestations, laboratory results, therapeutic regimen and prognosis. Results： The major clinical manifestations of these 5 POEMS syndrome patients were peripheral neuropathy 100%, endocrinopathy 100%, skin lesions 100%, organomegaly 80%, and positive M protein 60%, respectively. Four patients improved after treatment with prednisone or immunodepressants, and one patient aggravated when discharged from hospital. Only one patient remained improved at the end of the follow-up. Conclusions： The clinical manifestations of POEMS syndrome are rather complicated, with the progress in the diagnosis and treatment of the syndrome, the prognosis will be greatly improved.