Department of Hematology and Oncology, Zhongda Hospital, School of Medicine, Southeast University, Nanjing, People's Republic of China.
Hematology. 2023 Dec;28(1):2254556. doi: 10.1080/16078454.2023.2254556. Epub 2023 Sep 21.
Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder, exhibiting a more unfavorable prognosis than multiple myeloma. PCL is classified into pPCL and sPCL. Recently, the IMWG has recommended new PCL definition criteria, which require the presence of ≥5% circulating plasma cells in peripheral blood smears. Due to its low incidence, research on pPCL and sPCL is limited.
We conducted a retrospective study and analyzed clinical and cytogenetic data of pPCL and sPCL patients. Overall survival (OS) and progression-free survival (PFS) were assessed by the Kaplan-Meier method, and survival distributions were compared using the log-rank test.
This is a small cohort comprising 23 pPCL and 9 sPCL patients. Notably, sPCL patients showed a higher incidence of extramedullary infiltration and a higher percentage of bone marrow plasma cells (= 0.015 and 0.025, respectively). Although no significant difference was found between the two groups in OS and PFS, a trend emerged suggesting a superior survival outcome for pPCL patients, with a higher cumulative 1-year PFS rate (38.3% vs. 13.3%) and a lower early mortality rate (mortality rate at 3 months: 15% vs. 33%). We also suggested that pPCL patients carrying t(11;14) may have a longer median survival time than individuals with other cytogenetic abnormalities, but this was not confirmed due to the small sample size.
Our study revealed clinical and cytogenetic features of pPCL and sPCL patients according to the new diagnostic criteria. The findings suggested a generally better prognosis for pPCL than sPCL and the likelihood of t(11;14) translocation acting as a favorable prognostic factor in pPCL. It is important to note that our study had a limited sample size, which may lead to bias. We hope well-designed studies can be conducted to provide more results.
浆细胞白血病（PCL）是一种罕见且侵袭性的浆细胞疾病，其预后比多发性骨髓瘤更差。PCL 分为原发性浆细胞白血病（pPCL）和继发性浆细胞白血病（sPCL）。最近，IMWG 推荐了新的 PCL 定义标准，要求外周血涂片中有≥5%的循环浆细胞。由于发病率低，对 pPCL 和 sPCL 的研究有限。
我们进行了一项回顾性研究，分析了 pPCL 和 sPCL 患者的临床和细胞遗传学数据。通过 Kaplan-Meier 法评估总生存期（OS）和无进展生存期（PFS），使用对数秩检验比较生存分布。
这是一个小队列，包括 23 例 pPCL 和 9 例 sPCL 患者。值得注意的是，sPCL 患者骨髓浆细胞比例较高（=0.015 和 0.025），且骨髓外浸润发生率更高。尽管两组 OS 和 PFS 无显著差异，但 pPCL 患者的生存结果似乎更好，累积 1 年 PFS 率更高（38.3% vs. 13.3%），早期死亡率更低（3 个月死亡率：15% vs. 33%）。我们还发现携带 t(11;14)的 pPCL 患者的中位生存时间可能长于具有其他细胞遗传学异常的患者，但由于样本量较小，这一结果未得到证实。
根据新的诊断标准，本研究揭示了 pPCL 和 sPCL 患者的临床和细胞遗传学特征。研究结果表明，pPCL 的预后普遍优于 sPCL，并且 t(11;14)易位可能是 pPCL 的有利预后因素。需要注意的是，本研究的样本量有限，可能存在偏倚。我们希望能够开展设计良好的研究，提供更多结果。
