POEMS综合征31例临床分析

目的：报道31例POEMS综合征，结合文献探讨其临床特点，实验室检查，诊断标准并简述其可能发病机制和治疗。方法：把病人临床表现与国外文献比较，总结其特点。结果：POEMS综合征多见于中年男性，发病年龄28-71岁，平均49.5岁。临床表现以多发性周围神经病、脏器肿大、内分泌病、M-蛋白和皮肤改变为特征。常与骨硬化型骨髓瘤并存。随病程进展相继出现低热、多汗、视乳头水肿、红细胞增多症等。常因多发脏器血栓形成、肺感染、呼吸衰竭或心力衰竭死亡。骨髓放射学和内分泌学有独特表现。发病机制不详。激素疗法缓解症状。结论：我国病人发现骨髓瘤比例明显偏低，阳瘘、男性乳房增大、闭经等多见，其次为周围神经损害、肢端水肿、皮肤改变等。

Clinical analysis on 31 cases of POEMS syndrome

Objective: To report 31 cases of POEMS syndrome, a brief review was made about its clinical features,laboratory examination,diagnosis and its pathogenesis and treatment were elucidated.Methods: Clinical manefestations were compared with literature abrord, its characteristic was summarized.Results:POEMS syndrome often affects middle age of man, the age at onset ranged from 28 to 71 years,average, 49.5 years. Clinical display were characterized by a combination of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes.It usually exists with osteosclerotic myeloma.Developmental stagnation occured, followed by slight fever,hyperhidrosis,papilledema, polycythemia, et al. Most died of multiple thrombotic events, pneumonia, respiratory insufficiency or heart failure.Radiology and endocrinology have a unique style. Pathogenesis remaims unknown.Treatment with prednisolone have led to improvement.Conclusion:The patients with myeloma in our country are fewer than others. However, amenorrhoea in women, gynecomastia and impotence in men are more common than in other countries. Secondary, clinical features are polyneuropathy, edema and skin changes, et al.