POEMS综合征是一种由潜在的浆细胞肿瘤引起的危及生命的疾病。该综合征的主要标准包括多发性根神经炎、克隆性浆细胞紊乱、硬化性骨病变、血管内皮生长因子升高以及Castleman病的存在。次要特征包括器官肿大、内分泌功能障碍、皮肤特征改变、视盘水肿、血管外容量超负荷和血小板增多症。诊断POEMS综合征需要满足三个主要标准，其中两个必须包括多发性根神经炎和克隆性浆细胞紊乱，并且至少需要满足一个次要标准。由于该综合征的发病机制尚不完全清楚，风险分层仅限于临床表型而非具体的分子标记。风险因素包括低血清白蛋白、年龄、胸腔积液、肺动脉高压和估计的肾小球滤过率减低。对于有主导型浆细胞瘤的患者，放射治疗是一线治疗方法。有弥漫性硬化病变或播散性骨髓受累的患者应接受系统性治疗。皮质类固醇具有暂时性作用，但碱化剂和来那度胺是治疗的基石，前者作为低剂量常规治疗或作为干细胞移植的高剂量准备。沙利度胺和硼替佐米也具有活性，但需要权衡其加剧外周神经病变的风险和受益。基于病例系列研究，达瑞珠单抗联合疗法似乎也很有前景。及时识别并实施支持性护理措施和针对浆细胞的治疗是获得最佳效果的关键。© 2023 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.

POEMS syndrome is a life-threatening condition due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder, sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis.The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced estimated glomerular filtration rate.For those patients with a dominant plasmacytoma, first-line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement should receive systemic therapy. Corticosteroids are temporizing, but alkylators and lenalidomide are the mainstays of treatment, the former either in the form of low-dose conventional therapy or as high-dose conditioning for stem cell transplantation. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Daratumumab combinations also appear promising based on case series. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.© 2023 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.