关键词: Primary plasma cell leukemia cytogenetics prognosis secondary plasma cell leukemia treatment

Mesh : Humans Leukemia, Plasma Cell / diagnosis genetics Retrospective Studies Cytogenetics Multiple Myeloma Cytogenetic Analysis

来  源:   DOI:10.1080/16078454.2023.2254556

Abstract:
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要:
浆细胞白血病(PCL)是一种罕见的侵袭性浆细胞疾病,表现出比多发性骨髓瘤更不利的预后。PCL分为pPCL和sPCL。最近,IMWG推荐了新的PCL定义标准,这需要在外周血涂片中存在≥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,并分析了pPCL和sPCL患者的临床和细胞遗传学数据。通过Kaplan-Meier方法评估总生存期(OS)和无进展生存期(PFS),和生存分布使用对数秩检验进行比较。
这是一个由23名pPCL和9名sPCL患者组成的小队列。值得注意的是,sPCL患者的髓外浸润发生率较高,骨髓浆细胞百分比较高(分别为p=0.015和0.025)。尽管两组在OS和PFS上没有发现显著差异,出现了一种趋势,表明pPCL患者具有优越的生存结局,具有较高的1年累计PFS率(38.3%与13.3%)和较低的早期死亡率(3个月时的死亡率:15%vs.33%)。我们还表明,携带t(11;14)的pPCL患者可能比其他细胞遗传学异常的个体有更长的中位生存时间。但由于样本量小,这没有得到证实。
根据新的诊断标准,我们的研究揭示了pPCL和sPCL患者的临床和细胞遗传学特征。研究结果表明,pPCL的预后通常比sPCL更好,并且t(11;14)易位可能是pPCL的有利预后因素。值得注意的是,我们的研究样本量有限,这可能会导致偏见。我们希望精心设计的研究能够提供更多的结果。
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