Epidemiology of Plasma Cell Leukemia in the Czech Republic

Klin Onkol. 2019 Winter;32(1):47-51. doi: 10.14735/amko2019.

Abstract

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 dis-ease 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 us-ing The Czech National Cancer Registry (CNCR) as the basic source of data for the population-based evaluation of PCL epidemiology.

Results: Accord-ing to CNCR data, there were on average six newly dia-gnosed 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 dia-gnosis in the CNCR.

Conclusion: Data from registries such as the CNCR can provide useful information on epidemiology of various dis-eases. These data, however, have several limitations, such as dia-gnostic criteria and proper cod-ing of not only the dis-ease itself, but also its various forms. These limitations have to be taken into account dur-ing the process of results interpretation. Key words plasma cell leukemia -  epidemiology -  Czech National Cancer Registry (CNCR) -  Czech Republic.

Keywords: plasma cell leukemia -  epidemiology -  Czech National Cancer Registry (CNCR) -  Czech Republic.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Child, Preschool
  • Czech Republic / epidemiology
  • Female
  • Humans
  • Incidence
  • Infant
  • Infant, Newborn
  • Leukemia, Plasma Cell / epidemiology*
  • Male
  • Middle Aged
  • Young Adult