Klin Onkol 2019; 32(1): 47-51. DOI: 10.14735/amko201947.
Background: Plasma cell leukemia (PCL) is a rare but most aggressive form of monoclonal gammopathies. PCL is characterized by the presence of clonal plasma cells in peripheral blood. There are two forms of PCL – primary which presents de novo in patients with no evidence of previous multiple myeloma and secondary which is a leukemic transformation of relapsed or refractory disease in patients with previously recognized multiple myeloma. Materials and methods: This is the first study to provide information on PCL epidemiology in the Czech population using The Czech National Cancer Registry (CNCR) as the basic source of data for the population-based evaluation of PCL epidemiology. Results: According to CNCR data, there were on average six newly diagnosed cases of PCL and four deaths caused by PCL each year in the Czech Republic in the period 2000–2015. PCL incidence in the Czech Republic was reported at 0.57 per million in 2000–2015. We suppose that most reported cases of PCL are primary PCL because secondary PCL is a relapse of a previously reported myeloma and, in most cases, is not coded as an independent diagnosis in the CNCR. Conclusion: Data from registries such as the CNCR can provide useful information on epidemiology of various diseases. These data, however, have several limitations, such as diagnostic criteria and proper coding of not only the disease itself, but also its various forms. These limitations have to be taken into account during the process of results interpretation.
