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POEMS综合征:渗出性腹水和慢性周围神经病变的罕见病因。

POEMS syndrome: a rare cause of exudative ascites and chronic peripheral neuropathy.

作者信息

Al-Mayoof Omar, Al Sughaiyer Hana, Abuomar Wuroud, Khan Maria

机构信息

Infectious Disease Unit, Rashid Hospital, Dubai, UAE.

Internal Medicine Department, Rashid Hospital, Dubai, UAE.

出版信息

BMJ Case Rep. 2017 Jun 20;2017:bcr-2016-219022. doi: 10.1136/bcr-2016-219022.

Abstract

POEMS syndrome (Peripheral neuropathy, Organomegaly, Endocrinopathy, M protein, Skin changes) is a rare plasma cell disorder with multisystem involvementA 40-year-old man with a chronic history of unexplained peripheral neuropathy , presented with exudative ascites.He was found to have an incidental osteolytic lesion of the sacrum proven to be a plasmacytoma. Immunoelectrophoresis for monoclonal protein was negative.In addition, the patient was found to have hepatosplenomegaly, erectile dysfunction, hyperprolactinaemia and hypothyroidism.The patient, was given steroid along with lenalidomide, and showed a good clinical response.

摘要

POEMS综合征(周围神经病、脏器肿大、内分泌病、M蛋白、皮肤改变)是一种罕见的累及多系统的浆细胞疾病。一名有不明原因慢性周围神经病病史的40岁男性,出现渗出性腹水。他被偶然发现骶骨有溶骨性病变,经证实为浆细胞瘤。单克隆蛋白免疫电泳为阴性。此外,该患者还存在肝脾肿大、勃起功能障碍、高泌乳素血症和甲状腺功能减退。给予该患者类固醇及来那度胺治疗后,临床反应良好。

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POEMS Syndrome: Therapeutic Options.POEMS综合征:治疗选择
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