Bin Zhang1, XingWang Song, Bin Liang, QingHua Hou, ShuXiang Pu, Jian Rui Ying, Cong Gao. 1. Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and The Ministry of Education of China, Institute of Neuroscience and Department of Neurology, The Second Affiliated Hospital of GuangZhou Medical Universit, China. zhangbin19781130@yahoo.com.cn
Abstract
OBJECTIVE: POEMS syndrome is a unique clinical entity. It was described by the presence of several typical characteristics as paraproteinemia, polyneuropathy, organomegaly, endocrinopathy, and skin changes. Few people reported characteristics of Chinese POEMS patients. PATIENTS AND METHODS: Retrospective evaluation of Chinese patients with POEMS syndrome was carried out to reveal clinical features and compare with foreign series reported previously. In addition to typical characteristics, Chinese patients often were presented with extravascular volume overload (84%), papilledema (44%), bone lesions (41%), raynaud phenomenon (31%) and Clubbing (22%). RESULTS: Clinical laboratory tests found most patients had increased erythrosedimentation rate, hyperlipidemia, liver disorder and renal involvement. 70% patients had hypothyroidism, including overt hypothyroidism (6 patients) and subclinical hypothyroidism (13 patients). High prevalence of positive antimitochondrial antibody (ANA) and positive antineutrophil cytoplasmic antibody (ANCA) was the more common phenomenon. Chinese patients with bone damage in the incidence are lower than in the West and Japanese reports. CONCLUSIONS: In summary, our study demonstrate the POEMS syndrome diagnostic criteria proposed by Dispenzieri et al is also more high applicability in the Chinese population.At the time of diagnosis, we should not pay attention on the typical characteristics of the disease, but also on the changes in thyroid, liver, kidney function and lipid metabolism.
OBJECTIVE:POEMS syndrome is a unique clinical entity. It was described by the presence of several typical characteristics as paraproteinemia, polyneuropathy, organomegaly, endocrinopathy, and skin changes. Few people reported characteristics of Chinese POEMS patients. PATIENTS AND METHODS: Retrospective evaluation of Chinese patients with POEMS syndrome was carried out to reveal clinical features and compare with foreign series reported previously. In addition to typical characteristics, Chinese patients often were presented with extravascular volume overload (84%), papilledema (44%), bone lesions (41%), raynaud phenomenon (31%) and Clubbing (22%). RESULTS: Clinical laboratory tests found most patients had increased erythrosedimentation rate, hyperlipidemia, liver disorder and renal involvement. 70% patients had hypothyroidism, including overt hypothyroidism (6 patients) and subclinical hypothyroidism (13 patients). High prevalence of positive antimitochondrial antibody (ANA) and positive antineutrophil cytoplasmic antibody (ANCA) was the more common phenomenon. Chinese patients with bone damage in the incidence are lower than in the West and Japanese reports. CONCLUSIONS: In summary, our study demonstrate the POEMS syndrome diagnostic criteria proposed by Dispenzieri et al is also more high applicability in the Chinese population.At the time of diagnosis, we should not pay attention on the typical characteristics of the disease, but also on the changes in thyroid, liver, kidney function and lipid metabolism.