Xiaofeng Shi1, Shudong Hu2, Xianqiu Yu3, Qin Zhuang3, Min Luo3, Qian Jiang3, Lixia Wang3, Yilong Lu3, Xiaoming Fei3, Xiaodong Xi2, Yan Zhu4. 1. Department of Hematology, Affiliated Hospital of Jiangsu University, Zhenjiang, Jiangsu, China; Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. 2. Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China. 3. Department of Hematology, Affiliated Hospital of Jiangsu University, Zhenjiang, Jiangsu, China. 4. Department of Hematology, Affiliated Hospital of Jiangsu University, Zhenjiang, Jiangsu, China. Electronic address: zhuyan19591959@163.com.
Abstract
BACKGROUND: POEMS syndrome, a rare paraneoplastic disease, is related to multiple organs, multiple systems, and multiple disciplines and can be mistaken for other disorders. Consequently, the diagnoses are often delayed. In this work we studied the clinicopathologic characteristics of the POEMS syndrome to improve early diagnosis to prevent irreversible damage. PATIENTS AND METHODS: We conducted a clinicopathologic analysis of 9 cases of POEMS and made a differential diagnosis with related diseases. RESULTS: The patients with POEMS syndrome were shown to have complicated clinical characteristics, including peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma proliferative disorder, skin changes, extravascular volume overload, sclerotic bone lesions, thrombocytosis, and Castleman disease. POEMS syndrome shared many elements with other diseases and the key way to differentiate them was to determine whether there were other fundamental POEMS syndrome symptoms or signs. The level of M-protein in serum and plasma cells in bone marrow of POEMS patients was lower than that of patients with multiple myeloma (MM). Sclerotic bone lesions were a distinctive feature in patients with POEMS, compared with in those with MM. CONCLUSION: Some unique clinicopathologic characteristics of POEMS syndrome can be used for differential diagnosis. This study provides increased awareness of POEMS syndrome.
BACKGROUND:POEMS syndrome, a rare paraneoplastic disease, is related to multiple organs, multiple systems, and multiple disciplines and can be mistaken for other disorders. Consequently, the diagnoses are often delayed. In this work we studied the clinicopathologic characteristics of the POEMS syndrome to improve early diagnosis to prevent irreversible damage. PATIENTS AND METHODS: We conducted a clinicopathologic analysis of 9 cases of POEMS and made a differential diagnosis with related diseases. RESULTS: The patients with POEMS syndrome were shown to have complicated clinical characteristics, including peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma proliferative disorder, skin changes, extravascular volume overload, sclerotic bone lesions, thrombocytosis, and Castleman disease. POEMS syndrome shared many elements with other diseases and the key way to differentiate them was to determine whether there were other fundamental POEMS syndrome symptoms or signs. The level of M-protein in serum and plasma cells in bone marrow of POEMS patients was lower than that of patients with multiple myeloma (MM). Sclerotic bone lesions were a distinctive feature in patients with POEMS, compared with in those with MM. CONCLUSION: Some unique clinicopathologic characteristics of POEMS syndrome can be used for differential diagnosis. This study provides increased awareness of POEMS syndrome.