Chen Fei, Wang Yan, Huang Zhongxia, Qi Man, Chen Hong
Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chao-Yang Hospital, Capital Medical University, 5 Jingyuan Road, Shijingshan District, Beijing, 100043, China.
Department of Hematology, Aerospace Center Hospital, 15 Yuquan Road, Haidian District, Beijing, 100049, China.
World J Surg Oncol. 2025 May 31;23(1):210. doi: 10.1186/s12957-025-03851-w.
Anaplastic multiple myeloma (AMM) is a type of distinctive MM with a poor prognosis. AMM is mostly reported as individual cases, an accurate incidence and strict clinical definition are lacking.
Sixty-seven patients with AMM were identified and then analyzed in the clinical database of patients with MM from January 2017 to September 2024.
The incidence of AMM among patients with MM was 3.3%. The IgD type accounted for 11.9% of patients, with 40.3% and 53.7% kappa and lambda light chains, respectively. Plasmablasts with larger diameters and multinuclear variations accounted for 46.7% from bone marrow or extramedullary disease (EMD) based on pathologic morphology; the average Ki-67 was 64.8%. The incidence of EMD was 49.3%. Lactic dehydrogenase (LDH) was elevated in 44.8% of patients and 49.3% of patients were International Staging System (ISS) III. The frequencies of 1q21, t (4; 14), and t (14; 16) in high-risk genes were 60.5%, 39.5%, and 18.4%, respectively. Double and triple gene hits were detected in 36.8% and 13.2% of patients, respectively. Fifty-six patients received treatment with bortezomib-based regimens. The progression-free survival (PFS) and overall survival (OS) of AMM patients in the autologous stem cell transplantation (ASCT) group were prolonged compared to patients who did not undergo ASCT with a median PFS and OS of 5.0 versus 25.0 and 17.0 versus 36.0 months, respectively (P = 0.0246 and P = 0.0119, respectively). At the time of the last follow-up, 71.4% of patients had died with 77.5% experiencing disease progression. A high neutrophil-to-lymphocyte ratio (NLR) and no ASCT were also independent prognostic factors for AMM patients.
The incidence of AMM is rare. The characteristics of these AMM patients included extensive proliferation of plasmablasts and widespread EMD formation, AMM patients also exhibit more invasive clinical manifestations and a short survival. Patients who underwent sequential ASCT after receiving bortezomib-based regimens partially overcame the poor prognosis of AMM.
间变性多发性骨髓瘤(AMM)是一种独特的多发性骨髓瘤,预后较差。AMM大多作为个案报道,缺乏准确的发病率和严格的临床定义。
在2017年1月至2024年9月的多发性骨髓瘤患者临床数据库中识别并分析了67例AMM患者。
AMM在多发性骨髓瘤患者中的发病率为3.3%。IgD型占患者的11.9%,κ和λ轻链分别占40.3%和53.7%。根据病理形态学,直径较大且有多核变异的浆母细胞在骨髓或髓外疾病(EMD)中占46.7%;平均Ki-67为64.8%。EMD的发病率为49.3%。44.8%的患者乳酸脱氢酶(LDH)升高,49.3%的患者为国际分期系统(ISS)III期。高危基因中1q21、t(4;14)和t(14;16)的频率分别为60.5%、39.5%和18.4%。分别在36.8%和13.2%的患者中检测到双基因和三基因打击。56例患者接受了基于硼替佐米的方案治疗。与未接受自体干细胞移植(ASCT)的患者相比,ASCT组AMM患者的无进展生存期(PFS)和总生存期(OS)延长,中位PFS和OS分别为5.0个月对25.0个月和17.0个月对36.0个月(P分别为0.0246和0.0119)。在最后一次随访时,71.4%的患者死亡,77.5%的患者出现疾病进展。高中性粒细胞与淋巴细胞比值(NLR)和未进行ASCT也是AMM患者的独立预后因素。
AMM的发病率罕见。这些AMM患者的特征包括浆母细胞广泛增殖和广泛的EMD形成,AMM患者还表现出更具侵袭性的临床表现和较短的生存期。接受基于硼替佐米的方案后序贯ASCT的患者部分克服了AMM的不良预后。