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病例报告:DKRd方案治疗新诊断的POEMS综合征及文献综述

Case report: DKRd regimen in the treatment of newly diagnosed POEMS syndrome and literature review.

作者信息

Wang Jianchao, Liao Wensheng, Liu Zhongwen, Kong Dai

机构信息

Department of Surgery of Spine and Spinal Cord, Henan Provincial People's Hospital, Zhengzhou University People's Hospital, Zhengzhou, China.

Department of Hematology, Henan Provincial People's Hospital, Zhengzhou University People's Hospital, Zhengzhou, China.

出版信息

Front Oncol. 2024 Aug 1;14:1417380. doi: 10.3389/fonc.2024.1417380. eCollection 2024.

Abstract

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.

摘要

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。1980年由巴德威克提出,首字母缩略词POEMS描绘了该综合征的显著特征: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改变。POEMS综合征的患病率约为每10万人中有0.3例。由于其患病率低且前瞻性研究匮乏,目前的治疗方法很大程度上依赖于回顾性研究,并且围绕通常用于多发性骨髓瘤治疗的浆细胞定向疗法展开。本文介绍了使用DKRd(达雷妥尤单抗、卡非佐米、来那度胺和地塞米松)四联方案作为一线治疗的首例病例。随后进行诱导治疗,接着是自体造血干细胞移植。并对相关文献进行了全面综述。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/98a1/11324492/9fa7432d42f4/fonc-14-1417380-g001.jpg

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