Li Yating, Sun Zhengxu, Qu Xiaoyan
The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, China.
The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, China.
Transl Oncol. 2022 Aug;22:101465. doi: 10.1016/j.tranon.2022.101465. Epub 2022 Jun 6.
Extramedullary disease (EMD) is characterized by plasma cells outside of bone marrow in multiple myeloma (MM) patients, which results in an adverse prognosis. The cornerstone of treatment consists of combination therapy including proteasome inhibitors, immunomodulatory agents, steroids, followed by consolidative autologous hematopoietic stem cell transplantation in eligible patients. This review summarized the recent advances in the treatment of EMD. Bortezomib based therapy showed efficacy and was recommended to treat EMD. Marizomib had advantages in the treatment of central nervous system-multiple myeloma (CNS-MM) because of its good central nervous system penetrability. Immunomodulatory drugs such as lenalidomide and pomalidomide have been reported to be effective. Isatuximab and selinexor were also active. Based on the treatment experience of EMD in our department, we summarized treatment approach for EMD. However, the benefits of patients with EMD from the new era of novel drugs were limited. Novel drugs combination, monoclonal antibody, molecular targeted therapy, cellular immunotherapy and autologous stem cell transplantation (ASCT) are under investigation. Therapeutic studies and clinical trials specifically target EMD should be conducted. Hopefully, these treatment options for EMD will be demonstrated efficacy in the future.
髓外疾病（EMD）的特征是多发性骨髓瘤（MM）患者骨髓外存在浆细胞，这会导致预后不良。治疗的基石包括联合治疗，其中有蛋白酶体抑制剂、免疫调节剂、类固醇，随后对符合条件的患者进行巩固性自体造血干细胞移植。本综述总结了EMD治疗的最新进展。基于硼替佐米的治疗显示出疗效，被推荐用于治疗EMD。马立佐米因其良好的中枢神经系统穿透性，在治疗中枢神经系统多发性骨髓瘤（CNS-MM）方面具有优势。据报道，来那度胺和泊马度胺等免疫调节药物有效。isatuximab和selinexor也有活性。基于我科对EMD的治疗经验，我们总结了EMD的治疗方法。然而，EMD患者从新药新时代中获得的益处有限。新药联合、单克隆抗体、分子靶向治疗、细胞免疫治疗和自体干细胞移植（ASCT）正在研究中。应开展专门针对EMD的治疗研究和临床试验。有望这些EMD的治疗选择在未来能证明其疗效。
