POEMS综合征20例临床分析

Clinical analysis of 20 patients with POEMS syndrome

目的总结POEMS综合征的临床特点及治疗措施，旨在提高其诊治水平。方法对20例POEMS综合征患者的一般资料、临床表现、化验及辅助检查结果进行回顾性分析。19例患者使用糖皮质激素治疗，其中9例予静脉输注大剂量免疫球蛋白观察疗效：另1例患者单独使用了硫唑嘌呤。结果全部患者均有多发性神经病．其他临床表现包括：皮肤改变、内分泌病、皮肤水肿、脏器肿大；M蛋白阳性率为6／13。治疗后9例患者症状有所减轻。单用糖皮质激素及糖皮质激素合用免疫球蛋白的治疗效果无显著性差异。结论POEMS综合征是一种较少见的多系统损害综合征，诊断有一定困难，应对其加强认识，对疑诊病例需进行必要的检查，以免误诊；免疫球蛋白对其无明显疗效。

Objective To summarize the clinical features and therapeutic measures of POEMS syndrome （polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes）, in order to improve the level of diagnosis and treatment. Methods General data, clinical manifestations, tests and auxiliary examinations of 20 patients with POEMS syndrome were retrospectively analyzed. Nineteen of all the patients were treated with corticoids and 9 treated with additional infusion of high-dose intravenous immunoglobulin, the left one patient was treated only with azathioprine. Results Polyneuropathy was examined in all the 20 patients. Other clinical manifestations included hyperpigmented skin, cndocrinopathy, edema of skin and organomegaly. A total of 6/13 （positive rate） among all patients were complicated with M protein. No obvious differences were detected in therapeutic effects between patients treated with corticoids alone and those treated with the combined therapy of corticoids and immunoglobulin. Symptoms were alleviated in 9 case of all the patients. Conclusion POEMS syndrome is a comparatively rare multisystem disorder of obscure pathogenesis, which makes its diagnosis difficult. To avoid misdiagnosis, understanding of the disease should be intensified and necessary examinations should be undertaken for cases suspected to suffer from the disease. Up until now, high-dose intravenous immunoglobulin is of no obvious effect on POEMS syndrome.