Plasma Cell Leukemia –  the Forgotten Dis­ease
BACKGROUND
Plasma cell leukemia (PCL) is a rare dis-ease and possibly the most aggressive form of monoclonal gammopathy. It is classified into two forms -&#8202; primary PCL that occurs without a previously identifiable multiple myeloma stage, and secondary PCL that develops from previously dia-gnosed multiple myeloma. These two forms have different cytogenetic and molecular profiles, but both forms have an aggressive clinical course. Combinations of different therapeutic approaches includ-ing autologous stem cell transplantation and currently proteasome inhibitors and immunomodulatory drugs are used to treat PCL. Current dia-gnostic criteria, developed in the 1970s, may underestimate PCL prevalence; thus, prospective re-evaluation is be-ing considered.


PURPOSE
The aim of this study is to review all available information about PCL with an emphasis on dia-gnostics, treatment, and circulat-ing plasma cells features.


CONCLUSION
Although PCL is rare, it is quite a severe dis-ease. Current treatments us-ing the latest therapeutics have prolonged patient survival. However, due to the low incidence of PCL, information about the dis-ease 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 understand-ing of its pathogenesis. Key words plasma cell leukemia -&#8202; multiple myeloma -&#8202; plasma cells -&#8202; cytogenetics -&#8202; treatment This work was supported by grant NV18-03-00203. The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submited: 2. 11. 2018 Accepted: 18. 11. 2018.
['Žárska|M|M|', 'Vrábel|D|D|', 'Bezdekova|R|R|', 'Štork|M|M|', 'Jarošová|M|M|', 'Adam|Z|Z|', 'Krejčí|M|M|', 'Pour|L|L|', 'Ševčíková|S|S|']
D006801:Humans D007952:Leukemia, Plasma Cell D035583:Rare Diseases
plasma cell leukemia -&#8202 multiple myeloma -&#8202 plasma cells -&#8202 cytogenetics -&#8202 products or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.

Submited: 2. 11. 2018

Accepted: 18. 11. 2018 treatment

This work was supported by grant NV18-03-00203.

The authors declare they have no potential conflicts of interest concerning drugs