Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Hwasun, Republic of Korea.
Sungkyunkwan University School of Medicine, Samsung Medical Center, Seoul, Republic of Korea.
Blood Cancer J. 2022 Nov 21;12(11):157. doi: 10.1038/s41408-022-00755-w.
The International Myeloma Working Group has recently revised the diagnostic criteria for primary plasma cell leukemia (PCL) to circulating plasma cells (CPCs) ≥ 5% in a peripheral blood smear. The present study validated new criteria in patients with multiple myeloma or PCL diagnosed using the previous diagnostic criteria, who were administered immunomodulatory drugs or proteasome inhibitors as induction therapy. We analyzed the medical records of 1357 patients from eight hospitals in South Korea. The median age of the all patients was 64 years, and 187 (13.8%) had CPCs at diagnosis. Only 79 (5.8%) of the patients had ≥ 5% CPCs. The median overall survival (OS) of patients with CPCs ≥ 5% and ≥ 20% was similar, but had significantly inferior median progression-free survival (PFS) and median OS than those with CPCs < 5% (13.1 vs. 21.5 months, P < 0.001, and 21.5 vs. 60.9 months, P < 0.001, respectively). Primary PCL diagnosed using the revised criteria presented with higher total calcium levels and serum creatinine levels, lower platelet counts and frequent organomegaly and plasmacytoma at diagnosis. Univariate and multivariate analyses demonstrated that the presence of plasmacytoma and elevated serum β2-microglobulin were significantly associated with OS in primary PCL. In conclusion, the revised criterion of CPCs ≥ 5% in a peripheral blood smear is appropriate for PCL diagnosis.
国际骨髓瘤工作组最近修订了原发性浆细胞白血病(PCL)的诊断标准,将外周血涂片中循环浆细胞(CPCs)≥5%作为诊断标准。本研究在接受免疫调节剂或蛋白酶体抑制剂诱导治疗的多发性骨髓瘤或符合旧诊断标准的 PCL 患者中验证了新的标准。我们分析了来自韩国 8 家医院的 1357 名患者的病历。所有患者的中位年龄为 64 岁,187 名(13.8%)患者在诊断时有 CPCs。仅有 79 名(5.8%)患者的 CPCs≥5%。CPCs≥5%和≥20%的患者的中位总生存期(OS)相似,但无进展生存期(PFS)和 OS 明显短于 CPCs<5%的患者(13.1 与 21.5 个月,P<0.001;21.5 与 60.9 个月,P<0.001)。使用修订标准诊断的原发性 PCL 患者总钙和血清肌酐水平更高,血小板计数更低,更常出现器官肿大和浆细胞瘤。单因素和多因素分析表明,浆细胞瘤的存在和血清β2-微球蛋白升高与原发性 PCL 的 OS 显著相关。总之,外周血涂片 CPCs≥5%的新标准适用于 PCL 的诊断。