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目的 :探讨POMES综合征的临床特点 ,提高诊治水平。方法 :对 13例POEMS综合征临床资料进行回顾分析。结果 :本组临床特征与国外组基本一致。不同点为本组病例以周围神经病为突出表现 ,常伴植物神经损害 ;视乳头水肿、脾肿大多见 ,性腺机能减退较明显 ,甲状腺功能低下多见而临床症状不明显 ;淋巴结肿大少见 ;M蛋白检出率较低。结论 :本组临床特征与国外组基本相似 ,但有其特点 ,早期极易误诊 ,免疫抑制剂治疗有效。病因和发病机制尚不清楚。
Objective: To explore the clinical features of POMES syndrome and to improve the diagnosis and treatment. Methods: The clinical data of 13 POEMS syndromes were retrospectively analyzed. Results: The clinical features of this group are basically the same as those of foreign group. Differences in this group of patients with peripheral neuropathy as a prominent manifestation, often accompanied by autonomic nerve damage; papilledema, splenomegaly most common, hypogonadism more obvious, more common hypothyroidism and clinical symptoms were not obvious; lymphadenopathy rarely seen; M protein detection rate is low. Conclusion: The clinical features in this group are basically similar to those in foreign countries, but they are characterized by early misdiagnosis and effective immunosuppressive therapy. Etiology and pathogenesis is not clear.