Cause of death (COD) in POEMS (polyneuropathy, organomegaly, endocrinopathies, monoclonal protein and skin changes) syndrome is not well described. We investigated COD in patients with POEMS syndrome treated at Mayo Clinic between 2000 and 2022. Of the 89 deaths, 49 patients had known COD and were the subject of this study. Seventeen patients died of unrelated causes, while 32 patients (65%) died from causes related to POEMS syndrome including secondary malignancies like myelodysplastic syndrome and acute leukemia (n = 5) and complications from active therapy (n = 5). Notably, 19 patients died with a stereotypic syndrome we termed capillary leak phenotype (CLP), which was characterized by refractory ascites, effusions and/or anasarca that ultimately resulted in hypotension, renal failure and cardiopulmonary arrest. Alternate causes for these symptoms, such as cardiac and hepatic etiologies, were excluded. CLP as a COD was an earlier event with a median time from diagnosis to death of 2.5 years compared to 12.0 years for all other deceased patients (p = <0.0001). By definition, treatment of terminal CLP was unsuccessful with median survival of only 4 months after CLP onset. The driver of CLP is unknown, but recognition as an entity should allow for systematic study.

POEMS （多发性神经病、器官肿大、内分泌病、单克隆蛋白和皮肤变化） 综合征的死因 （COD） 没有得到很好的描述。我们调查了 2000 年至 2022 年间在妙佑医疗国际接受治疗的 POEMS 综合征患者的 COD。在 89 例死亡中，49 例患者已知 COD 并且是本研究的对象。17 名患者死于无关原因，而 32 名患者 （65%） 死于与 POEMS 综合征相关的原因，包括骨髓增生异常综合征和急性白血病等继发性恶性肿瘤 （n = 5） 和积极治疗并发症 （n = 5）。值得注意的是，19 例患者死于我们称之为毛细血管渗漏表型 （CLP） 的刻板综合征，其特征是难治性腹水、积液和/或吻合，最终导致低血压、肾衰竭和心肺骤停。排除了这些症状的其他原因，例如心脏和肝脏病因。CLP 作为 COD 是一个较早的事件，从诊断到死亡的中位时间为 2.5 年，而所有其他已故患者为 12.0 年 （p = <0.0001）。根据定义，终末 CLP 的治疗不成功，CLP 发病后中位生存期仅为 4 个月。CLP 的驱动因素尚不清楚，但作为一个实体的认可应该允许进行系统研究。