POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare plasma cell dyscrasia without standard front-line treatment. Merely, few studies have reported the responses and outcomes of bortezomib plus dexamethasone (BDex) in POEMS syndrome. In this study, a total of 69 patients (40 males) treated with front-line BDex were included. The median age at diagnosis was 50 years (range, 30–78 years). After a median of 9 cycles BDex (range 1–9), fifty-two (88.1%), thirty-two (46.4%), and forty-seven (71.2%) patients achieved the best neurologic response, hematological complete response, and serum vascular endothelial growth factor (VEGF) response, respectively. The extravascular overload, pulmonary hypertension, and renal impairment also substantially improved. No treatment-related death occurred. Two patients developed grade-1 bortezomib-induced peripheral neuropathy and were reversible after drug withdrawal. After a median follow-up of 22.5 months, the estimated 2-year overall survival and time to next treatment were 95.7% and 65.6%, respectively. In conclusion, the combination of bortezomib and dexamethasone is effective, with a high response rate and safety profile for patients with newly diagnosed POEMS syndrome.

POEMS（多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变）综合征是一种罕见的浆细胞恶液质，没有标准的一线治疗。只是，很少有研究报告硼替佐米加地塞米松 (BDex) 在 POEMS 综合征中的反应和结果。在这项研究中，共纳入了 69 名接受一线 BDex 治疗的患者（40 名男性）。诊断时的中位年龄为 50 岁（范围为 30-78 岁）。在中位 9 个周期的 BDex（范围 1-9）后，五十二名 (88.1%)、三十二名 (46.4%) 和四十七名 (71.2%) 患者获得了最佳的神经系统反应、血液学完全反应和血清血管内皮生长因子（VEGF）反应，分别。血管外超负荷、肺动脉高压和肾功能损害也显着改善。没有发生与治疗相关的死亡。两名患者出现了 1 级硼替佐米诱导的周围神经病变，并且在停药后是可逆的。中位随访 22.5 个月后，估计的 2 年总生存率和下次治疗时间分别为 95.7% 和 65.6%。总之，硼替佐米联合地塞米松是有效的，对新诊断的 POEMS 综合征患者具有较高的反应率和安全性。