Amyloidosis and Myeloma Unit, Department of Hematology, Hospital Clínic of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Internal Medicine, Division of Hematology, Mayo Clinic, Rochester, MN, USA.
Blood Cancer J. 2021 Dec 2;11(12):192. doi: 10.1038/s41408-021-00587-0.
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.
原发性浆细胞白血病（PCL）的预后一直很凶险，即便在过去几十年取得了进展。PCL 需要迅速识别，以便为这种超高风险疾病提供最有效的治疗。本文旨在根据其他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，重新审视 PCL 的诊断。我们可以确定两个回顾性系列，其中涉及外周血中应使用多少个循环浆细胞来定义 PCL 的问题。在 MM 患者中存在≥5%的循环浆细胞与之前定义的 PCL 具有相似的不良预后影响。因此，在其他诊断为有症状多发性骨髓瘤的患者中，应通过外周血涂片存在 5%或更多的循环浆细胞来定义 PCL。
