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.
国际骨髓瘤工作组近期修订了原发性浆细胞白血病的诊断标准，将外周血涂片中循环浆细胞比例≥5%纳入诊断依据。本研究旨在验证新标准在多发性骨髓瘤或采用旧标准诊断的原发性浆细胞白血病患者中的适用性，这些患者均接受免疫调节药物或蛋白酶体抑制剂作为诱导治疗。我们分析了韩国八家医院共1357例患者的医疗记录。所有患者的中位年龄为64岁，其中187例（13.8%）在诊断时存在循环浆细胞。仅有79例（5.8%）患者循环浆细胞比例≥5%。循环浆细胞比例≥5%与≥20%的患者中位总生存期相近，但其中位无进展生存期和中位总生存期均显著低于循环浆细胞比例<5%的患者（分别为13.1个月对21.5个月，P<0.001；21.5个月对60.9个月，P<0.001）。根据修订标准诊断的原发性浆细胞白血病患者初诊时表现为更高的血钙水平和血清肌酐水平、更低的血小板计数，以及更常见的器官肿大和浆细胞瘤。单变量与多变量分析显示，浆细胞瘤的存在和血清β2-微球蛋白升高与原发性浆细胞白血病患者的总生存期显著相关。综上所述，采用外周血涂片中循环浆细胞比例≥5%作为修订诊断标准适用于浆细胞白血病的诊断。
肿瘤(癌症)患者之家