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伴有双克隆丙种球蛋白病的POEMS综合征:一种罕见的关联。

POEMS Syndrome with Biclonal Gammopathy: A Rare Association.

作者信息

De Dibyendu, Halder Sandeepan, Datta Suvro Sankha

机构信息

Consultant, Department of Haematology, The Mission Hospital, Durgapur, West Bengal, India.

Consultant, Department of Pathology, The Mission Hospital, Durgapur, West Bengal, India.

出版信息

J Clin Diagn Res. 2017 Jul;11(7):XD12-XD13. doi: 10.7860/JCDR/2017/24800.10265. Epub 2017 Jul 1.

DOI:10.7860/JCDR/2017/24800.10265
PMID:28893029
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5583889/
Abstract

Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes (POEMS) syndrome is rare plasma cell dyscrasia with multisystem involvement. The name comes from the five characteristic features: Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. The presence of biclonal M band is a rare manifestation. Here, we are describing the cases of a 60-year-old lady, presented with bilateral pedal oedema and pericardial effusion and peripheral neuropathy. She also had hepatosplenomegaly, hyperpigmented rash and hypothyroidism and hyperparathyroidism. The serum protein electrophoresis and the immunofixation electrophoresis revealed two distinct monoclonal bands, immunoglobulin IgG kappa and IgA lambda. There was a mild increase in plasma cells and sclerotic bone lesion in pelvis. The POEMS syndrome is generally associated with lambda light chain restriction. The presence of biclonal gammopathy involving kappa and lambda is a rare manifestation. The pathogenic or prognostic role of different paraprotein is not known. Further studies are required to delineate such effect.

摘要

多神经病、器官肿大、内分泌病、M蛋白和皮肤改变(POEMS)综合征是一种罕见的累及多系统的浆细胞异常增殖性疾病。该名称源于五个特征:多神经病、器官肿大、内分泌病、M蛋白和皮肤改变。双克隆M带的出现是一种罕见表现。在此,我们描述了一名60岁女性的病例,她出现双侧足部水肿、心包积液和周围神经病变。她还伴有肝脾肿大、色素沉着性皮疹以及甲状腺功能减退和甲状旁腺功能亢进。血清蛋白电泳和免疫固定电泳显示出两条不同的单克隆带,即免疫球蛋白IgG κ和IgA λ。浆细胞有轻度增多,骨盆出现硬化性骨病变。POEMS综合征通常与λ轻链限制有关。涉及κ和λ的双克隆丙种球蛋白病的出现是一种罕见表现。不同副蛋白的致病或预后作用尚不清楚。需要进一步研究来阐明这种效应。

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本文引用的文献

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POEMS Syndrome with IgG-λ/IgA-κ Biclonal Gammopathy and Abnormal Serum Free Light Chain Ratio: a Case Report.伴有IgG-λ/IgA-κ双克隆丙种球蛋白病及异常血清游离轻链比值的POEMS综合征:一例报告
Ann Clin Lab Sci. 2015 Fall;45(6):702-6.
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