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目的报告1例以神经系统病变为首发症状,合并脏器肿大、内分泌系统功能紊乱及骨质增生的POMES综合征病例。方法回顾性分析POMES综合征的临床表现、骨髓活检结果、诊疗经过,结合文献对POMES综合征临床特点、诊断标准及治疗进行探讨。结果老年男性患者,临床表现为四肢无力伴麻木感、疼痛2个月余,尿轻链测定显示κ,λ链异常,B超检查示脏器肿大,肌电图检查示神经源性损害,PET-CT检查示有不同程度的骨质增生,骨髓涂片显示粒系、红系增生活跃。结论对于出现神经系统症状,合并脏器肿大、内分泌系统功能紊乱及骨质增生的患者应考虑POMES综合征可能。
Objective To report a case of POMES syndrome with neurological disease as the first symptom, complicated organ enlargement, endocrine system dysfunction and bone hyperplasia. Methods The clinical manifestations, bone marrow biopsy results, diagnosis and treatment of POMES syndrome were retrospectively analyzed. The clinical features, diagnostic criteria and treatment of POMES syndrome were discussed. Results In elderly male patients, clinical manifestations were limb weakness with numbness, pain more than 2 months, urinary light chain assay showed abnormalities of κ and λ chain, B-ultrasound showed organ enlargement, EMG showed neurogenic damage, PET-CT showed different degrees of bone hyperplasia, bone marrow smear showed granulocyte, erythroid hyperplasia. Conclusions POMES syndrome should be considered in patients with neurological symptoms, enlarged organ, endocrine system disorders and osteoarthritis.