Gao Shuang, Li Qihui, Dong Fei, Yang Ping, Chen Yingtong, Wang Jing, Wang Yanfang, Jing Hongmei
Department of Hematology, Lymphoma Center, Peking University, Third Hospital, Beijing, China.
Department of Hematology, Lymphoma Center, Peking University, Third Hospital, Beijing, China.
Leuk Res. 2022 Apr;115:106793. doi: 10.1016/j.leukres.2022.106793. Epub 2022 Jan 29.
The aim of study was to assess the clinical characteristics, survival outcomes of newly diagnosed multiple myeloma (NDMM) patients with extramedullary disease (EMD). We retrospectively reviewed and compared the clinical features and outcomes in 226 MM patients with or without EMD at their diagnosis. EMD patients were subsequently divided into bone-related (EMD-B) and soft-tissue related (EMD-S). EMD group was composed of 29.2% of the patients and the incidence of EMD-B and EMD-S was 19.0% and 10.2% respectively. EMD at diagnosis had greater prevalence of IGH/FGFR3 translocation (7.6% vs 2.5%, p = 0.028) and a worse objective response rate (75% vs 88.9%, p = 0.015). The overall survival (OS) of EMD and non-EMD patients were 44 months and 82 months respectively (p < 0.001)，and the progression-free survival were 24 months and 36 months respectively (p = 0.014). Multivariate analysis showed EMD at diagnosis, hypercalcemia, and elevated LDH were independent prognostic factors. Prevalence of anemia, elevated LDH, 1q21 + were greater in EMD-S and OS of EMD-S was significantly poorer (51months vs 26 months, p = 0.0067) compared with EMD-B. Autologous hematopoietic stem cell transplantation (auto-HSCT) could partly overcome the adverse effect of EMD.
本研究旨在评估新诊断的伴有髓外疾病（EMD）的多发性骨髓瘤（NDMM）患者的临床特征及生存结局。我们回顾性分析并比较了226例初诊时伴有或不伴有EMD的骨髓瘤患者的临床特征及结局。随后，EMD患者被分为骨相关（EMD-B）和软组织相关（EMD-S）两组。EMD患者占患者总数的29.2%，EMD-B和EMD-S的发生率分别为19.0%和10.2%。诊断时EMD患者IGH/FGFR3易位的发生率更高（7.6% 对2.5%，p = 0.028），客观缓解率更低（75% 对88.9%，p = 0.015）。EMD患者和非EMD患者的总生存期（OS）分别为44个月和82个月（p < 0.001），无进展生存期分别为24个月和36个月（p = 0.014）。多因素分析显示，诊断时的EMD、高钙血症和乳酸脱氢酶（LDH）升高是独立的预后因素。EMD-S患者贫血、LDH升高、1q21+的发生率更高，与EMD-B相比，EMD-S患者的OS明显更差（51个月对26个月，p = 0.0067）。自体造血干细胞移植（auto-HSCT）可部分克服EMD的不良影响。
