Plasma cell leukemia (PCL) is a rare and aggressive form of malignant plasma cell neoplasm, either presenting as primary PCL (pPCL) or secondary PCL (sPCL). The prognosis of PCL patients is poor, with a overall survival (OS) of 6-11 months and a high fatality rate within 1 month after diagnosis. Currently, there is no standard treatment for PCL, and the usual treatment is derived from the traditional chemotherapy regimens of multiple myeloma (MM). But the treatment is less effective and benefit to PCL patients. With the applications of autologous hematopoietic stem cell transplantation(auto-HSCT), allogeneic hematopoietic stem cell transplantation (allo-HSCT), immunomodulatory drugs (IMiD) represented by nalidomide, protease inhibitor (PI) represented by bortezomib, molecular targeted therapy and chimeric antigen receptor T-cell(CAR-T) immunotherapy, the survival of PCL patients has been improved significantly. This article summarizes the current status of traditional chemotherapy regimens in treatment of PCL, as well as the research progress of the aforementioned new treatment methods for PCL, in order to provide a basis for improving the survival and prognosis of PCL patients.
