文章：

浆细胞白血病的修订诊断标准：梅奥诊所研究结果及其与多发性骨髓瘤的结局比较

Revised diagnostic criteria for plasma cell leukemia: results of a Mayo Clinic study with comparison of outcomes to multiple myeloma

原文发布日期：2018-11-15

DOI: 10.1038/s41408-018-0140-1

类型: Article

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英文摘要：

The current definition of plasma cell leukemia (PCL)— ≥ 20% circulating plasma cells (CPCs) on peripheral smear and plasma cell count ≥ 2 × 109/L—may be too stringent. We reviewed outcomes of 176 multiple myeloma (MM) patients diagnosed between 1971 and 2016, and who had CPCs detectable at diagnosis, to determine whether a lower threshold could be used to diagnose PCL. Median overall survival (mOS) was 1.1 years (95% CI 0.8–1.4) and was similar between patients with < 5% (n = 54, mOS = 1.4 years [0.7–2.0]), 5–19% (n = 63, mOS = 1.1 years [0.7–1.4]), and ≥ 20% CPCs (n = 59, mOS = 1.1 years [0.7–1.5], p = 0.349). As survival was similar between those with 5–19% and ≥ 20% CPCs, we stratified patients by < 5% (mOS = 1.4 years [0.7–2.0]) and ≥ 5% CPCs (mOS = 1.1 years [0.8–1.4], p = 0.154). Outcomes of those with ≥ 5% CPCs were much poorer when compared with a cohort of MM patients diagnosed between 1971 and 2016, who did not have CPCs at diagnosis (n = 9724, mOS = 4.4 yrs [4.3–4.5], p < 0.001); survival was also lower in patients diagnosed after 2001 with ≥ 5% CPCs (n = 62, mOS = 1.4 years [0.8–2.5]) compared with patients with standard risk (n = 1326, mOS = 7.5 years [7.0–8.7]) and high-risk MM (n = 381, mOS = 4.3 years [3.5–4.9], p < 0.001). We therefore propose that the definition of PCL be revised to patients with ≥ 5% CPCs on peripheral blood smear, who otherwise meet diagnostic criteria for MM.

摘要翻译： 

目前浆细胞白血病（PCL）的定义——外周血涂片循环浆细胞（CPCs）≥20%且浆细胞计数≥2×10⁹/L——可能过于严格。我们回顾了1971年至2016年间确诊的176例在诊断时检测到CPCs的多发性骨髓瘤（MM）患者的预后，以确定是否可采用更低阈值诊断PCL。患者中位总生存期（mOS）为1.1年（95%置信区间0.8-1.4），且CPCs＜5%（54例，mOS=1.4年[0.7-2.0]）、5-19%（63例，mOS=1.1年[0.7-1.4]）与≥20%（59例，mOS=1.1年[0.7-1.5]）三组间无显著差异（p=0.349）。鉴于5-19%与≥20%CPCs患者生存期相近，我们将患者按＜5%（mOS=1.4年[0.7-2.0]）和≥5%CPCs（mOS=1.1年[0.8-1.4]）分层分析（p=0.154）。与1971-2016年间诊断时未检出CPCs的MM患者队列（9724例，mOS=4.4年[4.3-4.5]）相比，≥5%CPCs患者预后显著更差（p＜0.001）；2001年后确诊的≥5%CPCs患者（62例，mOS=1.4年[0.8-2.5]）与标准风险组（1326例，mOS=7.5年[7.0-8.7]）及高危MM组（381例，mOS=4.3年[3.5-4.9]）相比生存期也更低（p＜0.001）。因此我们建议修订PCL定义为：外周血涂片CPCs≥5%且符合MM诊断标准的患者。

原文链接：

Revised diagnostic criteria for plasma cell leukemia: results of a Mayo Clinic study with comparison of outcomes to multiple myeloma