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Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 2010-Aug

[Clinical analysis of 22 cases of POEMS syndrome].

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Jie Lin
Hong-Li Zhu

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This study was purposed to investigate the clinical characteristics of POEMS syndrome in China, the clinical data of 22 patients with POEMS syndrome admitted in Chinese PLA general hospital from April 1993 to April 2008 were analyzed retrospectively. The results showed that the average age of patients was 47.7 years old, and the male:female ratio was 2.67. Weakness was the most frequent initial symptom. Nervous system lesions were found in all cases, weakness and numbness were predominant. Electromyogram showed neurogenic damage. Cerebrospinal fluid pressure and protein level were often elevated. Monoclonal band could be seen in electrophoresis of cerebrospinal fluid. Enlargement of liver, spleen and lymph nodes were common. Endocrine diseases accounted for 95.5%, in which gonadal dysfunction was most common. The sensitive test in M protein examination was immunofixation electrophoresis with positive rate 92.9%. IgA level was higher than IgG, and λ-type light chain was the majority, but κ-type light chain appeared in a small number of patients. Changes in skin included hyperpigmentation, hirsutism and sweating. In conclusion, clinical characteristics of patients in this group were basically similar to that reported in home, but were some different from that reported in abroad including following indicators: cerebrospinal fluid protein level, incidence of hepatomegaly and splenomegaly, serum protein level, light chain κ and λ level, incidence of peripheral edema, ascites, pleural effusion and platelet count.

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