Objective: to analyze the effect of circulating plasma cells(CPC) on the prognosis of patients with multiple myeloma(MM) in the era of new drugs, and to explore the new definition standard of primary plasma cell leukemia(pPCL).
Methods: The clinical data of 321 patients with newly diagnosed MM and 21 patients with pPCL admitted to our hospital from January 2014 to May 2022 were retrospectively analyzed. According to the proportion of CPC in peripheral blood smears, all patients were divided into 4 groups: CPC 0% group(211 cases), CPC 1%-4% group(69 cases), CPC 5%-19% group(41 cases) and CPC≥20% group(21 cases). The clinical features of patients in each group were compared and the prognosis fators was analyzed.
Results: The median OS of the four groups were 44.5,21.3,24.6 and 12.8 months, respectively. Among them, 295 patients(86.3%) were treated with new drugs, and the median OS of the four groups were not reached, 26.7, 24.6 and 14.9 months, respectively. As the survival curves of CPC 5%-19% group and CPC≥20% group were similar, the patients were divided into CPC<5% group and CPC≥5% group, the median OS of CPC<5% group was better than that in CPC≥5% (43.5 vs 22.3 months, P<0.001). In addition, the median OS of patients in the CPC 1%-4% group was also significantly lower than that in the CPC 0% group and similar to that in the CPC≥5% group. Multivariate analysis showed that 1%-4% CPC was an independent risk factor for the OS of patients with CPC<5%. The patients with CPC<5% were stratified by R-ISS staging, and the OS of R-ISS stage Ⅰ or stage Ⅱ with 1%-4% CPC was similar to that of R-ISS stage Ⅲ. The newly defined pPCL patients showed increased tumor load and obvious invasive characteristics. Multivariate analysis showed no independent prognostic factors for pPCL, and high-risk cytogenetic abnormalities(HRCA) had no significant effect on the prognosis.
Conclusion: The validity of IMWG's new pPCL definition standard was verified, and it was found that the survival of MM with 1%-4% CPC also is poor and the prognosis is very close to pPCL. In addition, the newly defined pPCL has unique clinical and biological characteristics.
题目: 循环浆细胞对多发性骨髓瘤患者预后的影响分析.
目的: 分析新药时代循环浆细胞(CPC)对多发性骨髓瘤(MM)患者预后的影响，并探讨原发性浆细胞白血病(pPCL)的新定义标准.
方法: 回顾性分析2014年1月至2022年5月本院收治的321例新诊断MM患者和21例pPCL患者的临床资料。全体患者根据外周血涂片中CPC比例分为CPC 0%(211例)、CPC 1%-4%(69例)、CPC 5%-19%(41例)和CPC≥20%组(21例)共4组，比较各组患者的临床特征及进行预后分析.
结果: 4组的中位OS分别为44.5、21.3、24.6和12.8个月。其中295例(86.3%)患者接受了新药治疗，4组患者的中位OS分别为未达到、26.7、24.6、14.9个月。由于CPC 5%-19%组和CPC≥20%组的生存曲线相近，将患者分为CPC<5%组和CPC≥5%组，CPC<5%组中位OS优于CPC≥5%组(43.5 vs 22.3个月，P<0.001)。此外，CPC 1%-4%组患者的中位OS同样显著低于CPC 0%组，且与CPC≥5%组相近。多因素分析结果显示，伴1%-4% CPC是影响CPC<5%组患者OS的独立危险因素。应用R-ISS分期对CPC<5%组患者进行分层，伴1%-4% CPC的R-ISSⅠ期或Ⅱ期患者的OS与R-ISSⅢ期相近。分析新定义下的pPCL患者，表现出肿瘤负荷增加和明显侵袭性特征。多因素分析未发现pPCL的独立预后因素，高危细胞遗传学异常(HRCA)对预后无明显的影响.
结论: 验证了IMWG新的pPCL定义标准的有效性，伴1%-4% CPC的MM同样具有较差的生存，预后非常接近pPCL。此外，新定义下的pPCL具有独特的临床及生物学特征.
Keywords: circulating plasma cells; multiple myeloma; plasma cell leukemia; prognostic factors.