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Incidence, characteristics, and outcome of solitary plasmacytoma and plasma cell leukemia. Population-based data from the Swedish Myeloma Register

Nahi, Hareth; Genell, Anna; Wålinder, Göran; Uttervall, Katarina; Juliusson, Gunnar LU ; Karin, Forsberg; Hansson, Markus LU ; Svensson, Ronald; Linder, Olle and Carlson, Kristina, et al. (2017) In European Journal of Haematology 99(3). p.216-222
Abstract

Solitary plasmacytoma (SP) and plasma cell leukemia (PCL) are uncommon (3-6%) types of plasma cell disease. The risk of progression to symptomatic multiple myeloma (MM) is probably important for the outcome of SP. PCL is rare and has a dismal outcome. In this study, we report on incidence and survival in PCL/SP, and progression to MM in SP, using the prospective observational Swedish Multiple Myeloma Register designed to document all newly diagnosed plasma cell diseases in Sweden since 2008. Both solitary bone plasmacytoma (SBP) (n=124) and extramedullary plasmacytoma (EMP) (n=67) have better overall survival (OS) than MM (n=3549). Progression to MM was higher in SBP than in EMP (35% and 7% at 2 years, respectively), but this did not... (More)

Solitary plasmacytoma (SP) and plasma cell leukemia (PCL) are uncommon (3-6%) types of plasma cell disease. The risk of progression to symptomatic multiple myeloma (MM) is probably important for the outcome of SP. PCL is rare and has a dismal outcome. In this study, we report on incidence and survival in PCL/SP, and progression to MM in SP, using the prospective observational Swedish Multiple Myeloma Register designed to document all newly diagnosed plasma cell diseases in Sweden since 2008. Both solitary bone plasmacytoma (SBP) (n=124) and extramedullary plasmacytoma (EMP) (n=67) have better overall survival (OS) than MM (n=3549). Progression to MM was higher in SBP than in EMP (35% and 7% at 2 years, respectively), but this did not translate into better survival in EMP. In spite of treatment developments, the OS of primary PCL is still dismal (median of 11 months, 0% at 5 years). Hence, there is a great need for diagnostic and treatment guidelines as well as prospective studies addressing the role for alternative treatment options, such as allogeneic stem cell transplantation and monoclonal antibodies in the treatment of PCL.

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publication status
published
subject
keywords
Multiple myeloma, Plasma cell neoplasms
in
European Journal of Haematology
volume
99
issue
3
pages
216 - 222
publisher
Wiley-Blackwell
external identifiers
  • scopus:85021440860
  • wos:000408148200003
ISSN
0902-4441
DOI
10.1111/ejh.12907
language
English
LU publication?
yes
id
47144aa0-dc6d-4f4b-8197-d284805ae2f1
date added to LUP
2017-08-22 11:55:07
date last changed
2017-09-18 11:42:33
@article{47144aa0-dc6d-4f4b-8197-d284805ae2f1,
  abstract     = {<p>Solitary plasmacytoma (SP) and plasma cell leukemia (PCL) are uncommon (3-6%) types of plasma cell disease. The risk of progression to symptomatic multiple myeloma (MM) is probably important for the outcome of SP. PCL is rare and has a dismal outcome. In this study, we report on incidence and survival in PCL/SP, and progression to MM in SP, using the prospective observational Swedish Multiple Myeloma Register designed to document all newly diagnosed plasma cell diseases in Sweden since 2008. Both solitary bone plasmacytoma (SBP) (n=124) and extramedullary plasmacytoma (EMP) (n=67) have better overall survival (OS) than MM (n=3549). Progression to MM was higher in SBP than in EMP (35% and 7% at 2 years, respectively), but this did not translate into better survival in EMP. In spite of treatment developments, the OS of primary PCL is still dismal (median of 11 months, 0% at 5 years). Hence, there is a great need for diagnostic and treatment guidelines as well as prospective studies addressing the role for alternative treatment options, such as allogeneic stem cell transplantation and monoclonal antibodies in the treatment of PCL.</p>},
  author       = {Nahi, Hareth and Genell, Anna and Wålinder, Göran and Uttervall, Katarina and Juliusson, Gunnar and Karin, Forsberg and Hansson, Markus and Svensson, Ronald and Linder, Olle and Carlson, Kristina and Björkstrand, Bo and Kristinsson, Sigurdur Y and Mellqvist, Ulf-Henrik and Blimark, Cecilie and Turesson, Ingemar},
  issn         = {0902-4441},
  keyword      = {Multiple myeloma,Plasma cell neoplasms},
  language     = {eng},
  number       = {3},
  pages        = {216--222},
  publisher    = {Wiley-Blackwell},
  series       = {European Journal of Haematology},
  title        = {Incidence, characteristics, and outcome of solitary plasmacytoma and plasma cell leukemia. Population-based data from the Swedish Myeloma Register},
  url          = {http://dx.doi.org/10.1111/ejh.12907},
  volume       = {99},
  year         = {2017},
}