Ji Min, Jin Shumin, Zang Shaolei, Li Peng, Lu Fei, Zhao Chuanli, Zhang Chunqing, Ji Chunyan, Ye Jingjing
Department of Hematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China.
Front Oncol. 2024 Dec 3;14:1449945. doi: 10.3389/fonc.2024.1449945. eCollection 2024.
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome is a paraneoplastic syndrome associated with an underlying plasma cell neoplasm. According to the current diagnostic criteria for POEMS syndrome, the presence of characteristic polyneuropathy and clonal plasma cell disorder are required for diagnosis. We report a case of a Castleman disease variant of POEMS syndrome without monoclonal protein (M protein) expression, which presented with polyneuropathy, organomegaly, endocrinopathy, skin lesions, and sclerotic bone lesions. The patient was treated with lenalidomide and dexamethasone (RD), after which her symptoms improved. The findings in this case suggest that the diagnostic criteria for POEMS syndrome might require reconsideration.
POEMS(多发性神经病、器官肿大、内分泌病、单克隆蛋白、皮肤改变)综合征是一种与潜在浆细胞肿瘤相关的副肿瘤综合征。根据目前POEMS综合征的诊断标准,诊断需要存在特征性的多发性神经病和克隆性浆细胞疾病。我们报告一例无单克隆蛋白(M蛋白)表达的POEMS综合征的Castleman病变异型病例,该病例表现为多发性神经病、器官肿大、内分泌病、皮肤病变和硬化性骨病变。患者接受来那度胺和地塞米松(RD)治疗后症状改善。该病例的发现提示POEMS综合征的诊断标准可能需要重新考虑。