原发性浆细胞白血病治疗进展

Advances in the treatment of primary plasma cell leukemia

摘要: 原发性浆细胞白血病（primary plasma cell leukemia，pPCL）是一种罕见、高度侵袭性的浆细胞恶性肿瘤。目前pPCL缺乏标准治疗方案，其整体治疗策略可以参照高危多发性骨髓瘤（multiple myeloma，MM）的思路。近年来，蛋白酶体抑制剂、免疫调节剂以及单克隆抗体、嵌合抗原受体T细胞免疫疗法（chimeric antigen receptor T-cell immunotherapy，CAR-T）、抗体偶联药物等靶向药物、免疫治疗与干细胞移植的联合大幅提高了MM的疗效，同时为pPCL的治疗提供了新的选择。本文就近年来相关进展进行综述。

Abstract: Primary plasma cell leukemia (pPCL) is a rare and very aggressive plasma cell malignancy. Even with currently available regimens for treating high-risk multiple myeloma (MM), the treatment of PCL remains challenging. The outcomes of MM have improved in recent years because of the use of proteasome inhibitors (PIs), immunomodulatory drugs (IMIDs), monoclonal antibodies, chimeric antigen receptor T-cell immunotherapy (CAR-T), and autologous stem cell transplantation. Therefore, novel regimens and stem cell transplantation are currently being investigated and will hopefully improve the prognosis of pPCL. In this review, we focus on advances in the treatment of PCL.