Oyama Takashi, Taoka Kazuki, Chiba Akira, Masamoto Yosuke, Ikemura Masako, Honda Akira, Maki Hiroaki, Kurokawa Mineo
Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
Department of Pathology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
Int J Hematol. 2023 Mar;117(3):463-467. doi: 10.1007/s12185-022-03459-w. Epub 2022 Oct 6.
The standard therapies for polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome are radiation therapy, high-dose chemotherapy followed by autologous stem cell transplantation, and lenalidomide combined with dexamethasone. Daratumumab was reported to be effective for treatment-naive and relapsed POEMS syndrome, but treatment options for relapsed POEMS syndrome with poor prognostic factors or cytogenetic abnormalities have not been established due to a lack of studies in these patients. Here, we describe a case of relapsed POEMS syndrome with bone plasmacytoma harboring a newly detected 17p deletion after high-dose chemotherapy followed by autologous stem cell transplantation and radiation therapy in a male patient. He was successfully treated with daratumumab plus lenalidomide and dexamethasone (Dara-Rd). Dara-Rd could be effective in relapsed POEMS syndrome with 17p deletion, which is known as a poor cytogenetic abnormality in multiple myeloma. This report may broaden the application of Dara-Rd for POEMS syndrome.
多神经病、器官肿大、内分泌病、单克隆蛋白血症和皮肤改变(POEMS)综合征的标准治疗方法是放射治疗、大剂量化疗后进行自体干细胞移植,以及来那度胺联合地塞米松。据报道,达雷妥尤单抗对初治和复发的POEMS综合征有效,但由于缺乏针对这些患者的研究,对于具有不良预后因素或细胞遗传学异常的复发POEMS综合征的治疗方案尚未确立。在此,我们描述了一例男性患者,在接受大剂量化疗后进行自体干细胞移植和放射治疗后,复发的POEMS综合征合并骨浆细胞瘤,且新检测到17p缺失。他接受达雷妥尤单抗联合来那度胺和地塞米松(Dara-Rd)治疗成功。Dara-Rd可能对伴有17p缺失的复发POEMS综合征有效,17p缺失在多发性骨髓瘤中是一种已知的不良细胞遗传学异常。本报告可能会拓宽Dara-Rd在POEMS综合征中的应用。