CAJ | 학술논문

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例患者均有周围神经病变,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.
목적 분석POEMS종합정적림상표현여치료.방법 회고성분석란주군구총의원2002년11월지2012년3월수치적12례POEMS종합정환자림상표현、실험실검사급치료방법병복습유관문헌,치료방법포괄지지치료、단용발니송、좌선분병안산담개연합발니송급사리도알치료등.결과 12례환자균유주위신경병변,발생솔100％ (12/12),간비종대발생솔66.7％(8/12),내분비이상발생솔83.3％(10/12),고M단백양성발생솔58.3％ (7/12),피부손해발생솔100％ (12/12),증상복잡다양.결론 본병위소견다계통손해질병,상유다발성주위신경병변、장기종대、내분비장애、M단백혈증화피부개변등증상화체정.림상응인진분석、검사,이제고림상검출솔.
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 retrospectively 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 organomegaly (8/12),endocrinopathy (10/12),monoclona gammopathy (7/12) and skin changes(12/12).Patients could benefit from the treatment of methylprednisolone and immunosuppressant.Conclusion POEMS syndrome is a rare disease with multiorgan involvement including polyneuropathy,organomegaly,endocrinopathy,M-proteins and skin changes.