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目的:探讨POEMS综合征临床特点。方法:回顾性分析10例POEMS综合征患者的临床资料。结果:POEMS综合征具有5大主要表现:周围神经病、脏器肿大、内分泌障碍、M蛋白以及皮肤损害。对孤立性病灶治疗可采取放疗和/或手术切除;激素、免疫抑制剂治疗效果差者可试用三苯氧胺进行治疗,也可采用大剂量化疗后自体外周血造血干细胞移植。结论:POEMS综合征临床表现复杂多样,提高认识可减少误诊;POEMS综合征可行放疗、化疗、手术及激素治疗,也可大剂量化疗后自体外周血造血干细胞移植。
Objective: To investigate the clinical features of POEMS syndrome. Methods: The clinical data of 10 patients with POEMS syndrome were retrospectively analyzed. Results: POEMS syndrome has five main manifestations: peripheral neuropathy, organ enlargement, endocrine disorders, M protein and skin lesions. Treatment of orphan lesion may be treated with radiotherapy and / or surgical excision. Patients with poor therapeutic effect of hormones and immunosuppressive drugs may be treated with tamoxifen or autologous peripheral blood stem cell transplantation may be used after high-dose chemotherapy. Conclusions: The clinical manifestations of POEMS syndrome are complex and diverse, and raising awareness can reduce the misdiagnosis. POEMS syndrome may be treated with radiotherapy, chemotherapy, surgery and hormone therapy, and may also be autologous peripheral blood stem cell transplantation after high-dose chemotherapy.