INTRODUCTION The definition of primary plasma cell leukemia (pPCL) has been revised from ≥20% to ≥5% circulating plasma cells (CPC). However, the precise prognosis associated with CPC remains controversial. This study aimed to investigate prognostic biomarkers for myeloma patients based on CPC presence. METHODS A comprehensive analysis was conducted on 309 consecutive patients diagnosed with either multiple myeloma or pPCL, utilizing peripheral blood smears stained with Wright-Giemsa. RESULTS Patients were grouped by CPC percentage: 0% (221, 71.5%), 1-4% (49, 15.9%), 5-19% (16, 5.2%), ≥20% (23, 7.4%). CPC >5% correlated with unfavorable characteristics, including anemia, renal dysfunction, and advanced International Staging System. Common cytogenetic abnormalities such as 1q21 amplification, 17p deletion, and Myc rearrangement were prevalent among CPC-positive patients. Median progression-free survival (PFS) and overall survival (OS) were shorter in patients with CPC ≥5% (29.47 vs. 10.03 months; 64.10 vs. 12.30 months). Additionally, PFS and OS were shorter in CPC-positive patients without autologous hematopoietic stem cell transplantation (ASCT) and those with response < partial remission to the first-line regimen. Furthermore, an association emerged between soft tissue-related extramedullary disease and inferior PFS, while Myc rearrangement correlated with abbreviated OS. CONCLUSION Biological characteristics displayed greater aggressiveness in patients with positive CPC, leading to significantly shorter PFS and OS. The presence of CPC, ASCT, and overall response rate were independent prognostic factors. While no new threshold for pPCL with CPCs is proposed, Myc rearrangements and CPC positivity could serve as ultra-high-risk factors for multiple myeloma.

中文翻译：

---

新标准定义的多发性骨髓瘤和原发性浆细胞白血病中循环浆细胞百分比的预后意义。


简介 原发性浆细胞白血病 (pPCL) 的定义已从循环浆细胞 (CPC) ≥20% 修订为≥5%。然而，与 CPC 相关的精确预后仍存在争议。本研究旨在研究基于 CPC 存在的骨髓瘤患者的预后生物标志物。方法 利用 Wright-Giemsa 染色的外周血涂片，对 309 名连续诊断为多发性骨髓瘤或 pPCL 的患者进行了全面分析。结果 患者按 CPC 百分比分组：0% (221, 71.5%)、1-4% (49, 15.9%)、5-19% (16, 5.2%)、≥20% (23, 7.4%)。 CPC > 5% 与不利特征相关，包括贫血、肾功能障碍和先进的国际分期系统。 CPC 阳性患者普遍存在常见的细胞遗传学异常，如 1q21 扩增、17p 缺失和 Myc 重排。 CPC ≥5% 的患者中位无进展生存期 (PFS) 和总生存期 (OS) 较短（29.47 个月与 10.03 个月；64.10 个月与 12.30 个月）。此外，未接受自体造血干细胞移植 (ASCT) 的 CPC 阳性患者以及对一线治疗方案有反应 < 部分缓解的患者的 PFS 和 OS 较短。此外，软组织相关的髓外疾病与较差的 PFS 之间存在关联，而 Myc 重排与缩短的 OS 相关。结论 CPC 阳性患者的生物学特征显示出更大的攻击性，导致 PFS 和 OS 显着缩短。 CPC、ASCT 的存在和总体缓解率是独立的预后因素。虽然没有提出 pPCL 与 CPC 的新阈值，但 Myc 重排和 CPC 阳性可能作为多发性骨髓瘤的超高风险因素。