文章：

原发性浆细胞白血病：国际骨髓瘤工作组依据外周血浆细胞百分比达成的共识定义

Primary plasma cell leukemia: consensus definition by the International Myeloma Working Group according to peripheral blood plasma cell percentage

原文发布日期：2021-12-02

DOI: 10.1038/s41408-021-00587-0

类型: Article

开放获取: 是

英文摘要：

Primary plasma cell leukemia (PCL) has a consistently ominous prognosis, even after progress in the last decades. PCL deserves a prompt identification to start the most effective treatment for this ultra-high-risk disease. The aim of this position paper is to revisit the diagnosis of PCL according to the presence of circulating plasma cells in patients otherwise meeting diagnostic criteria of multiple myeloma. We could identify two retrospective series where the question about what number of circulating plasma cells in peripheral blood should be used for defining PCL. The presence of ≥5% circulating plasma cells in patients with MM had a similar adverse prognostic impact as the previously defined PCL. Therefore, PCL should be defined by the presence of 5% or more circulating plasma cells in peripheral blood smears in patients otherwise diagnosed with symptomatic multiple myeloma.
 

摘要翻译： 

原发性浆细胞白血病（PCL）的预后持续严峻，即使在近几十年取得进展后仍如此。对于这种极高危疾病，需及时识别并启动最有效的治疗。本立场文件旨在根据符合多发性骨髓瘤诊断标准的患者外周血中循环浆细胞的存在情况，重新探讨PCL的诊断标准。通过回顾两项系列研究数据，我们探讨了外周血中循环浆细胞的界定比例对PCL诊断的意义。研究发现，多发性骨髓瘤患者外周血中≥5%的循环浆细胞与既往定义的PCL具有相似的预后不良影响。因此，对于已确诊症状性多发性骨髓瘤的患者，若外周血涂片显示循环浆细胞比例达5%或以上，应定义为PCL。

原文链接：

Primary plasma cell leukemia: consensus definition by the International Myeloma Working Group according to peripheral blood plasma cell percentage