Abstract：
BACKGROUND: 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.
CONCLUSIONS: 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.
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.
CONCLUSIONS: 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.
摘要：
背景：原发性浆细胞白血病（pPCL）的定义已从≥20％修订为≥5％循环浆细胞（CPC）。然而,与CPC相关的确切预后仍存在争议.这项研究旨在研究基于CPC存在的骨髓瘤患者的预后生物标志物。
方法：对309例连续诊断为多发性骨髓瘤或pPCL的患者进行了综合分析，利用Wright-Giemsa染色的外周血涂片。
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,肾功能不全,和先进的国际分期系统。常见的细胞遗传学异常，如1q21扩增，17p删除，和Myc重排在CPC阳性患者中普遍存在。CPC≥5%患者的中位无进展生存期（PFS）和总生存期（OS）较短（29.47vs.10.03个月；64.10与12.30个月）。此外,未进行自体造血干细胞移植（ASCT）的CPC阳性患者和一线方案缓解<部分缓解者的PFS和OS较短。此外，软组织相关的髓外疾病和下PFS之间出现关联，而Myc重排与缩写的OS相关。
结论：CPC阳性患者的生物学特征表现出更大的侵袭性，导致显著缩短PFS和OS。中国共产党的存在，ASCT,总有效率是影响预后的独立因素。虽然没有提出具有CPC的pPCL的新阈值，但Myc重排和CPC阳性可作为多发性骨髓瘤的超高风险因素.
方法：对309例连续诊断为多发性骨髓瘤或pPCL的患者进行了综合分析，利用Wright-Giemsa染色的外周血涂片。
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,肾功能不全,和先进的国际分期系统。常见的细胞遗传学异常，如1q21扩增，17p删除，和Myc重排在CPC阳性患者中普遍存在。CPC≥5%患者的中位无进展生存期（PFS）和总生存期（OS）较短（29.47vs.10.03个月；64.10与12.30个月）。此外,未进行自体造血干细胞移植（ASCT）的CPC阳性患者和一线方案缓解<部分缓解者的PFS和OS较短。此外，软组织相关的髓外疾病和下PFS之间出现关联，而Myc重排与缩写的OS相关。
结论：CPC阳性患者的生物学特征表现出更大的侵袭性，导致显著缩短PFS和OS。中国共产党的存在，ASCT,总有效率是影响预后的独立因素。虽然没有提出具有CPC的pPCL的新阈值，但Myc重排和CPC阳性可作为多发性骨髓瘤的超高风险因素.
