Current Status on Management of Primary Plasma Cell Leukemia.

IF 4.7 2区 医学 Q1 ONCOLOGY Current Oncology Reports Pub Date : 2024-09-01 Epub Date: 2024-07-02 DOI:10.1007/s11912-024-01563-0
Zimu Gong, Meera Khosla, Sreeraj Vasudevan, Meera Mohan
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Abstract

Purposeof review: Plasma Cell Leukemia (PCL) is a very rare and highly aggressive form of plasma cell dyscrasia. This review seeks to evaluate the outcomes of PCL in the context of combination novel agent therapy and stem cell transplant (SCT) protocols.

Recent findings: The diagnostic criteria for PCL have now evolved to include patients with 5% circulating PC. While management remains challenging, the incorporation of novel agent-based induction regimen has significantly improved early mortality and reduced attrition of patients proceeding to SCT. In recent prospective clinical trials, patients with PCL demonstrated an overall response rates of 69% to 86%, with progression-free and overall survival ranging from 13.8 to 15.5 months and 24.8 to 36.3 months, respectively. B-cell lymphoma 2 (BCL2) inhibitors, such as venetoclax present a targeted intervention opportunity for patients with PCL with t(11;14). Dedicated clinical trials tailored to PCL are crucial, integrating newer therapies in the frontline setting to further optimize responses and enhance overall outcomes.

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原发性浆细胞白血病的管理现状。
综述目的:浆细胞白血病(PCL)是一种非常罕见、侵袭性极强的浆细胞异常。本综述旨在评估PCL在新药联合治疗和干细胞移植(SCT)方案中的疗效:PCL的诊断标准现已发展到包括5%循环PC的患者。虽然治疗仍具有挑战性,但基于新型制剂的诱导方案已显著改善了早期死亡率,并减少了进行SCT患者的流失。在最近的前瞻性临床试验中,PCL 患者的总体反应率为 69% 至 86%,无进展生存期和总生存期分别为 13.8 至 15.5 个月和 24.8 至 36.3 个月。Venetoclax等B细胞淋巴瘤2(BCL2)抑制剂为t(11;14)型PCL患者提供了靶向干预的机会。专门针对 PCL 的临床试验至关重要,它将更新的疗法整合到一线治疗中,以进一步优化反应并提高总体疗效。
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来源期刊
CiteScore
8.50
自引率
0.00%
发文量
187
审稿时长
6-12 weeks
期刊介绍: This journal aims to review the most important, recently published clinical findings in the field of oncology. By providing clear, insightful, balanced contributions by international experts, the journal intends to serve all those involved in the care of those affected by cancer. We accomplish this aim by appointing international authorities to serve as Section Editors in key subject areas, such as cancer prevention, leukemia, melanoma, neuro-oncology, and palliative medicine. Section Editors, in turn, select topics for which leading experts contribute comprehensive review articles that emphasize new developments and recently published papers of major importance, highlighted by annotated reference lists. An international Editorial Board reviews the annual table of contents, suggests articles of special interest to their country/region, and ensures that topics are current and include emerging research. Commentaries from well-known figures in the field are also provided.
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