Klin Onkol 2019; 32(1): 40-46. DOI: 10.14735/amko201940.
Background: Plasma cell leukemia (PCL) is a rare disease and possibly the most aggressive form of monoclonal gammopathy. It is classified into two forms – primary PCL that occurs without a previously identifiable multiple myeloma stage, and secondary PCL that develops from previously diagnosed multiple myeloma. These two forms have different cytogenetic and molecular profiles, but both forms have an aggressive clinical course. Combinations of different therapeutic approaches including autologous stem cell transplantation and currently proteasome inhibitors and immunomodulatory drugs are used to treat PCL. Current diagnostic criteria, developed in the 1970s, may underestimate PCL prevalence; thus, prospective re-evaluation is being considered. Purpose: The aim of this study is to review all available information about PCL with an emphasis on diagnostics, treatment, and circulating plasma cells features. Conclusion: Although PCL is rare, it is quite a severe disease. Current treatments using the latest therapeutics have prolonged patient survival. However, due to the low incidence of PCL, information about the disease is very limited and comes mostly from small retrospective studies. Further studies of PCL are needed, because new information could increase in patient survival and our understanding of its pathogenesis
