研究动态
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新IMWG定义标准下原发和继发浆细胞白血病的临床和细胞遗传学特点:一项回顾性研究。

Clinical and cytogenetic characteristics of primary and secondary plasma cell leukemia under the new IMWG definition criteria: a retrospective study.

发表日期:2023 Dec
作者: Jiaheng Guan, Jinlong Ma, Baoan Chen
来源: Bone & Joint Journal

摘要:

浆细胞白血病(PCL)是一种罕见且具有侵袭性的浆细胞异常,其预后不如多发性骨髓瘤。PCL被分为pPCL和sPCL两类。最近,IMWG推荐了新的PCL定义标准,要求外周血涂片中循环浆细胞的存在率≥5%。由于其发病率低,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患者的临床及细胞遗传学数据。采用Kaplan-Meier方法评估总生存期(OS)和无进展生存期(PFS),并使用对数秩检验比较生存分布。该研究共纳入了23例pPCL和9例sPCL患者。值得注意的是,sPCL患者表现出更高的骨髓外浸润发生率和骨髓浆细胞百分比(p=0.015和0.025)。虽然两组在OS和PFS方面没有显著差异,但趋势显示pPCL患者有更好的生存结局,具有更高的累积1年PFS率(38.3% vs. 13.3%)和更低的早期死亡率(3个月的死亡率:15% vs. 33%)。我们还指出,携带t(11;14)的pPCL患者可能比其他细胞遗传学异常的个体具有更长的中位生存时间,但由于样本量较小,这一结果没有得到证实。我们的研究揭示了根据新的诊断标准对pPCL和sPCL患者的临床和细胞遗传学特征。研究结果表明,与sPCL相比,pPCL的预后普遍较好,并且t(11;14)易位可能是pPCL的有利预后因素。需要注意的是,我们的研究样本量有限,可能存在偏差。希望能进行设计良好的研究,提供更多的结果。
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 (p = 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.