Khwaja Jahanzaib, Keh Ryan, Smyth Duncan, Lunn Michael Peter, D'Sa Shirley, Sive Jonathan
Department of Haematology University College London Hospitals London UK.
Centre for Neuromuscular Disease National Hospital for Neurology and Neurosurgery, Queen Square London UK.
EJHaem. 2022 Jun 5;3(3):1021-1024. doi: 10.1002/jha2.492. eCollection 2022 Aug.
POEMS syndrome is a rareparaneoplastic disorder driven by an underlying low level plasma cell dyscrasiaand associated with elevated serum vascular endothelial growth factor (VEGF). Dueto its rarity, there are no internationally agreed standards of care, with verylimited data to guide management in the relapse setting. Agents used in myelomaare rational choices and have been employed. Daratumumab has been reported intwo case studies with lenalidomide-dexamethasone, one in the upfront and one inthe relapsed setting. We are the first to report here three cases ofdaratumumab-bortezomib-dexamethasone (DVd) use in relapsed POEMS postautologous stem cell transplant with good VEGF and clinical responses. Our casesadd to the literature on efficacy of daratumumab and are the first to report onits safe use with bortezomib in relapsed POEMS. It should be considered as aclinical option, in patients not responding to conventional first linetherapies.
POEMS综合征是一种罕见的副肿瘤性疾病，由潜在的低水平浆细胞发育异常驱动，并与血清血管内皮生长因子（VEGF）升高相关。由于其罕见性，目前尚无国际公认的治疗标准，在复发情况下指导治疗的数据非常有限。用于骨髓瘤的药物是合理的选择且已被应用。在两项病例研究中报告了达雷妥尤单抗联合来那度胺-地塞米松的情况，一项是一线治疗，另一项是复发治疗。我们在此首次报告3例自体干细胞移植后复发的POEMS综合征患者使用达雷妥尤单抗-硼替佐米-地塞米松（DVd）方案，VEGF和临床反应良好。我们的病例补充了关于达雷妥尤单抗疗效的文献，并且是首次报告其在复发的POEMS综合征中与硼替佐米联合安全使用的情况。对于对传统一线治疗无反应的患者，应将其视为一种临床选择。
