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[多发性神经病、器官肿大、内分泌病、M蛋白、皮肤改变:POEMS综合征]

[Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes: the POEMS syndrome].

作者信息

Lesić A, Simić A, Elezović I, Apostolski S, Vidaković A, Milović I

机构信息

Medical School, Belgrade.

出版信息

Srp Arh Celok Lek. 1993 Jan-Feb;121(1-2):39-40.

PMID:8202822
Abstract

We present a case of an unusual multi-systematic disorder whose cardinal signs were severe progressive sensorimotor polyneuropathy, hepatomegaly, endocrinopathy, plasma cell dyscrasia with osteosclerotic bone lesions and M-protein production as well as skin lesions (termed POEMS Syndrome) whose pathophysiology is still unknown, as well as its relationship with myeloma multiplex. The patient, 67 years old, had a history of progressive weakness and numbness of the lower legs, clinically revealed as sensomotor polyneuropathy, hepatomegaly, IgG lambda monoclonal protein in the serum, endocrine abnormalities and skin lesions. The final and definite diagnosis was established by open, surgical biopsy of the second lumbal vertebra.

摘要

我们报告一例罕见的多系统疾病,其主要症状为严重的进行性感觉运动性多神经病、肝肿大、内分泌病、伴有骨硬化性骨病变和M蛋白产生的浆细胞异常增生以及皮肤病变(称为POEMS综合征),其病理生理学尚不清楚,以及它与多发性骨髓瘤的关系。患者为67岁男性,有小腿进行性无力和麻木的病史,临床检查发现为感觉运动性多神经病、肝肿大、血清中IgG λ单克隆蛋白、内分泌异常和皮肤病变。最终通过对第二腰椎进行开放性手术活检确诊。

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