De Dibyendu, Halder Sandeepan, Datta Suvro Sankha
Consultant, Department of Haematology, The Mission Hospital, Durgapur, West Bengal, India.
Consultant, Department of Pathology, The Mission Hospital, Durgapur, West Bengal, India.
J Clin Diagn Res. 2017 Jul;11(7):XD12-XD13. doi: 10.7860/JCDR/2017/24800.10265. Epub 2017 Jul 1.
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.
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。