POEMS综合征9例临床分析

CLINICAL ANALYSIS OF 9 PATIENTS WITH POEMS SYNDROME

目的探讨POEMS综合征的临床特征和诊断治疗。方法对9例POEMS综合征患者临床资料、相关检查及治疗方法进行回顾性研究。结果主要临床特征:多发性周围神经病、脏器肿大,内分泌改变,单克隆浆细胞异常,皮肤改变,骨破坏,水肿是POEMS的主要表现。予强的松联合环磷酰胺治疗,有效率50%。结论POEMS综合征是一种少见的多脏器损害疾病,诊断较难。临床上需对可疑病例需进行密切观察及必要的检查,以免误诊。POEMS综合征患者主要治疗包括放疗、皮质激素、烷化物治疗,还包括大剂量化疗、外周血液干细胞移植。

Objective To evaluate the clinical features and therapies of POEMS syndrome. Methods The clinical materials of 9 patients with POEMS syndrome were analyzed retrospectively. Results The major clinical features are shown as follow： polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes, bone lesion and anemia. 50% patients are benefit from the treatment of prednisone and cyciophosphamide. Conclusion POEMS syndrome was a rare disease with multi organ involvement, which made its diagnosis difficult. So suspected cases should be given close observation and taken necessary examination to avoid misdiagnosis. The mainstays of therapy for patients with POEMS include irradiation, corticosteroids, and alkylator-based therapy, including high-dose chemotherapy with peripheral blood stem cell transplantation.