Plasma Cell Leukemia – the Forgotten Disease

Czech version

Authors: M. Žárska ¹; D. Vrábel ¹; R. Bezdekova ²; M. Štork ³; M. Jarošová ³; Z. Adam ³; M. Krejčí ³; L. Pour ³; S. Ševčíková ^1,2
Authors‘ workplace: Babákova myelomová skupina, Ústav patologické fyziologie, LF MU, Brno ¹; Oddělení klinické hematologie, FN Brno ²; Interní hematologická a onkologická klinika LF MU a FN Brno ³
Published in: Klin Onkol 2019; 32(1): 40-46
Category: Review

Overview

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.

Key words

plasma cell leukemia – multiple myeloma – plasma cells – cytogenetics – 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

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