文章：

浆细胞白血病临床视角：单中心经验分析

A Clinical Perspective on Plasma Cell Leukemia: A Single-Center Experience

原文发布日期：5 June 2024

DOI: 10.3390/cancers16112149

类型: Article

开放获取: 是

英文摘要：

Circulating plasma cells (CPCs) are detected in most multiple myeloma (MM) patients, both at diagnosis and on relapse. A small subset, plasma cell leukemia (PCL), represents a different biology and has a poor prognosis. In this retrospective analysis, we evaluated patients with primary (pPCL, n = 35) or secondary (sPCL, n = 49), with ≥5% CPCs and a smaller subset with lower CPCs of 1–4% (n = 20). The median age was 61 years; 45% were men and 54% were Black. High-risk cytogenetics were found in 87% and extramedullary disease in 47%. For the entire cohort, 75% received a proteasome inhibitor, 70% chemotherapy, 54% an immunomodulatory drug, 24% a daratumumab-based regimen and 26% an autologous stem cell transplant (ASCT). The treatments marginally improved the overall survival (OS) for pPCL vs. sPCL (13 vs. 3.5 monthsp= 0.002). However, the 5-year survival for the whole cohort was dismal at 11%. High-risk cytogenetics, low platelets, extramedullary disease and high LDH were independently associated with poor outcomes. Further research is urgently needed to expand the treatment options and improve the outcomes in PCL.

摘要翻译： 

循环浆细胞（CPCs）在大多数多发性骨髓瘤（MM）患者中均可检测到，无论是在初诊时还是复发时。其中一小部分患者发展为浆细胞白血病（PCL），其生物学特性不同且预后较差。本次回顾性分析中，我们评估了原发性（pPCL，n = 35）或继发性（sPCL，n = 49）PCL患者，这些患者的CPCs比例≥5%，另有一小部分患者CPCs比例较低（1-4%，n = 20）。患者中位年龄为61岁；45%为男性，54%为黑人。87%的患者存在高危细胞遗传学异常，47%伴有髓外病变。在整个队列中，75%的患者接受了蛋白酶体抑制剂治疗，70%接受化疗，54%使用免疫调节药物，24%接受达雷妥尤单抗为基础的治疗方案，26%进行了自体干细胞移植（ASCT）。与sPCL相比，pPCL患者的治疗仅略微改善了总生存期（OS）（13个月 vs. 3.5个月，p=0.002）。然而，整个队列的5年生存率仅为11%，预后极差。高危细胞遗传学异常、低血小板计数、髓外病变和高乳酸脱氢酶水平均独立与不良预后相关。亟需进一步研究以拓展PCL的治疗选择并改善患者预后。

原文链接：

A Clinical Perspective on Plasma Cell Leukemia: A Single-Center Experience