Primary plasma cell leukemia

Primary plasma cell leukemia (PPCL) is the most aggressive disorder among plasma cell malignancies, with new diagnostic criteria recently established by the International Myeloma Working Group. Studies have shown that PPCL patients receiving a combination of novel agents, but not eligible for transplantation, may have a median survival up to 2 years, extended to 3 years or more in those undergoing transplant procedures. These findings remain unsatisfactory, particularly if compared to progresses obtained in multiple myeloma. An extended overview of current biological, clinical, prognostic and therapeutic aspects of PPCL, including ongoing and close to start clinical trials, is presented. Furthermore, updated guidelines for the management of PPCL and practical recommendations are provided, in the context of current knowledge about this disease, also looking at possible future perspectives to ameliorate the outcome of these patients. Primary plasma cell leukemia still remains an unmet clinical need. Notwithstanding, some not negligible progresses has been recently achieved. The EMN panel strongly support ongoing and planned clinical trials, as well as biological studies based on novel technologies, strategies and treatment options that could represent breakthroughs we have been waiting for too long.