POEMS综合征临床分析

目的分析POEMS综合征的临床表现与治疗。方法回顾性分析兰州军区总医院2002年11月至2012年3月收治的12例POEMS综合征患者临床表现、实验室检查及治疗方法并复习有关文献,治疗方法包括支持治疗、单用泼尼松、左旋苯丙氨酸氮芥联合泼尼松及沙利度胺治疗等。结果12例患者均有周同神经病变,发生率100％（12／12）,肝脾肿大发生率66．7％（8／12）,内分泌异常发生率83．3％（10／12）,高M蛋白阳性发生率58．3％（7／12）,皮肤损害发生率100％（12／12）,症状复杂多样。结论本病为少见多系统损害疾病,常有多发性周围神经病变、脏器肿大、内分泌障碍、M蛋白血症和皮肤改变等症状和体征。临床应认真分析、检查,以提高临床检出率。

Clinical analysis of patients with POEMS syndrome and literature review

Objective To evaluate the clinica features and therapies of POEMS syndrome. Methods The clinical manifestations, laboratory tests of 12 patients with POEMS syndrome were analyzed retro- spectively from November 2002 to March 2012. Treatment options included supportive treatment, prednisone alone, melphalan, prednisone and thalidomide therapy. Results All 12 patients had peripheral polyneuropathy. Other major clinical features included organmnegaly （ 8/12 ）, endocrinopathy （ 10/12 ）, monoclona gammopathy （7/12） and skin changes（12/12）. Patients could benefit from the treatment of methylprednisolone and immuno- suppressant. Conclusion POEMS syndrome is a rare disease with multiorgan involvement including polyneuropathy, organomegaly, endocrinopathy,M-proteins and skin changes.