Thomas J, Maramattom B V, Varghese J, Kuruvilla P M
Department of Rheumatology, Lourdes Hospital, Kochi, Kerala, India.
Department of Neurology, Lourdes Hospital, Kochi, Kerala, India.
J Postgrad Med. 2016 Jul-Sep;62(3):205-7. doi: 10.4103/0022-3859.166181.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is characterized by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and one or more of the following features: Osteosclerotic myeloma, Castleman's disease (angiofollicular lymph node hyperplasia), increased levels of serum vascular endothelial growth factor (VEGF), organomegaly, endocrinopathy, edema, typical skin changes, and papilledema. [1] Solitary plasmacytomas most frequently occur in the bone but can also be found in soft tissues (extramedullary plasmacytoma). [2] We report a case that showed a rare association of POEMS syndrome with a plasmacytoma of the clivus.
多神经病、器官肿大、内分泌病、单克隆蛋白血症和皮肤改变（POEMS）综合征的特征是存在单克隆浆细胞疾病、周围神经病以及以下一种或多种特征：骨硬化性骨髓瘤、卡斯特曼病（血管滤泡性淋巴结增生）、血清血管内皮生长因子（VEGF）水平升高、器官肿大、内分泌病、水肿、典型皮肤改变和视乳头水肿。[1]孤立性浆细胞瘤最常发生于骨骼，但也可出现在软组织中（髓外浆细胞瘤）。[2]我们报告了一例显示POEMS综合征与斜坡浆细胞瘤罕见关联的病例。
