POEMS 综合征是一种罕见的多系统副肿瘤综合征，由浆细胞异常引起。 POEMS 由巴德威克于 1980 年创造，首字母缩写词 POEMS 描述了该综合征的独特特征：周围神经病变、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤变化。 POEMS 综合征的患病率约为每 10 万人中 0.3 人。由于其患病率低且缺乏前瞻性研究，目前的治疗方法很大程度上取决于回顾性研究，并围绕通常用于多发性骨髓瘤治疗的浆细胞定向疗法的使用。本文介绍了利用 DKRd 四药联合方案（达雷木单抗、卡非佐米、来那度胺和地塞米松）作为一线治疗的开创性案例。随后通过诱导治疗和自体造血干细胞移植取得成功。对相关文献进行了全面回顾。版权所有 © 2024 Wang、Liao、Liu 和 Kong。

POEMS syndrome, characterized as a rare multisystem paraneoplastic syndrome, arises from plasma cell abnormalities. Coined by Bardwick in 1980, the acronym POEMS delineates the distinctive features of the syndrome: Peripheral nerve Lesions, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes. The prevalence of POEMS syndrome stands at approximately 0.3 per 100,000 individuals. Owing to its low prevalence and the paucity of prospective studies, current treatment approaches largely hinge on retrospective studies and revolve around the use of plasma cell-directed therapy typically used in multiple myeloma treatments. This article presents the pioneering case of utilizing a four-drug combination regimen of DKRd (daratumumab, carfilzomib, lenalidomide, and dexamethasone) as a first-line treatment. This is succeeded by induction therapy and subsequently, autologous hematopoietic stem cell transplantation. A comprehensive review of related literature is conducted.Copyright © 2024 Wang, Liao, Liu and Kong.