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目的:探讨新药时代原发性浆细胞白血病（pPCL）的预后因素。方法:回顾性收集2011年至2022年就诊于首都医科大学附属北京朝阳医院血液科66例pPCL患者的临床资料，分析其预后因素。结果:66例pPCL患者中位发病年龄为59（29~79）岁。中位总生存（OS）期为19.0（95% CI 10.4~27.6）个月，中位无进展生存（PFS）期为11.0（95% CI 6.5~15.6）个月。治疗后最佳疗效≥非常好的部分缓解（VGPR）患者的中位OS期和PFS期均显著长于疗效≤部分缓解（PR）患者（中位OS期：33.0个月对6.0个月， P<0.001；中位PFS期：16.0个月对3.0个月， P<0.001）。接受自体造血干细胞移植患者的OS期较未接受患者显著延长（49.0个月对6.0个月， P=0.002），PFS期也有延长趋势（19.0个月对8.0个月， P=0.299）。接受维持治疗患者的中位OS期、PFS期较未接受维持治疗患者显著延长（中位OS期：56.0个月对4.0个月， P<0.001；中位PFS期：20.0个月对2.0个月， P<0.001）。多因素分析结果表明，高钙血症是影响pPCL患者OS的独立危险因素（ HR=3.204，95% CI 1.068~9.610， P=0.038）；接受维持治疗（ HR=0.075，95% CI 0.022~0.253， P<0.001）、治疗后疗效≥VGPR（ HR=0.175，95% CI 0.048~0.638， P=0.008）是影响pPCL患者OS的独立保护因素。 结论:在新药时代，高钙血症、接受维持治疗、治疗后疗效≥VGPR是pPCL的独立预后因素。

Objective:To explore the prognostic factors of primary plasma cell leukemia (pPCL) in the era of novel agents.Methods:The clinical data of 66 patients with pPCL treated at the Department of Haematology, Beijing Chao-Yang Hospital, Capital Medical University from 2011 to 2022 were retrospectively collected to analyze their prognostic factors.Results:Among the 66 patients with pPCL, the median age was 59 (range: 29-79) years. The median overall survival (OS) duration was 19.0 (95% CI 10.4-27.6) months, and the median progression-free survival (PFS) duration was 11.0 (95% CI 6.5-15.6) months. The median OS and PFS were significantly longer in patients with the best post-treatment response of very good partial remission (VGPR) or better than in patients with a response of partial remission (PR) or worse (median OS: 33.0 months vs 6.0 months, P<0.001; median PFS: 16.0 months vs 3.0 months, P<0.001). OS was significantly longer in patients who underwent autologous hematopoietic stem cell transplantation than in those who did not undergo transplantation (49.0 months vs 6.0 months, P=0.002), and there was a trend toward a longer PFS in patients who underwent transplantation than in those who did not undergo transplantation (19.0 months vs 8.0 months, P=0.299). The median OS and PFS were significantly longer in patients who received maintenance therapy than in those who did not receive maintenance therapy (median OS: 56.0 months vs 4.0 months, P<0.001; median PFS: 20.0 months vs 2.0 months, P<0.001). Multivariate analysis showed that hypercalcemia was an independent risk factor ( HR=3.204, 95% CI 1.068-9.610, P=0.038) for patients with pPCL, while receiving maintenance therapy ( HR=0.075, 95% CI 0.022-0.253, P<0.001) and post-treatment response of VGPR or better ( HR=0.175, 95% CI 0.048-0.638, P=0.008) were independent protective factors for patients with pPCL. Conclusions:In the era of novel agents, hypercalcemia, receiving maintenance therapy, and post-treatment response of VGPR or better are independent prognostic factors for pPCL.