Department of Medicine, Division of Hematology, Stanford Cancer Center, 875 Blake Wilbur Drive, Room 2329, Stanford, CA 94305-5821, USA.
Expert Rev Hematol. 2010 Oct;3(5):543-9. doi: 10.1586/ehm.10.52.
Plasma cell leukemia (PCL) is a rare and aggressive plasma cell dyscrasia. Patients with PCL have a very poor prognosis with median survival measured in months. PCL can present de novo or following a prodrome of plasma cell myeloma. Patients with PCL tend to present with aggressive clinical features, such as extramedullary disease, bone marrow failure, advanced stage disease and expression of distinct immunophenotypic markers, such as lack of CD56 and presence of CD20. Historically, the treatment of PCL has primarily been palliative, with only a small minority of patients achieving a durable remission. The impact of newer agents, such as bortezomib and lenalidomide, in conjunction with autologous and allogeneic stem cell transplantation is uncertain, but emerging data suggest that use of these modalities may help improve the poor prognosis of patients with PCL.
浆细胞白血病（PCL）是一种罕见且侵袭性的浆细胞恶性肿瘤。PCL 患者的预后非常差，中位生存期以月计。PCL 可以是原发性的，也可以是在浆细胞骨髓瘤前期出现。PCL 患者往往表现出侵袭性的临床特征，如髓外疾病、骨髓衰竭、晚期疾病和表达独特的免疫表型标志物，如缺乏 CD56 和存在 CD20。历史上，PCL 的治疗主要是姑息性的，只有极少数患者能够获得持久缓解。新型药物，如硼替佐米和来那度胺，联合自体和异基因干细胞移植的疗效尚不确定，但新出现的数据表明，这些治疗方法的应用可能有助于改善 PCL 患者的不良预后。
