Plasma cell leukemia

Plasma cell leukemia (PCL) is a rare and aggressive form of multiple myeloma (MM). It is characterized by the presence of a large number of circulating clonal plasma cells. It is comprised of two forms: (1) primary PCL (pPCL) which originate de novo with no prior history of MM and (2) secondary PCL (sPCL) where patients with a prior history of MM develop a leukemic phase of their MM. The natural history of either form of PCL is quite aggressive compared with MM. The incorporation of novel agent therapies and high-dose chemotherapy followed by stem cell rescue has made modest improvement in survival outcomes of patients with PCL over the last decade. Future, clinical trials incorporating various combinations of novel and experimental therapeutics are still warranted for the management of this disease.