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

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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 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.

Detaljer

Författare
  • Hareth Nahi
  • Anna Genell
  • Göran Wålinder
  • Katarina Uttervall
  • Gunnar Juliusson
  • Forsberg Karin
  • Markus Hansson
  • Ronald Svensson
  • Olle Linder
  • Kristina Carlson
  • Bo Björkstrand
  • Sigurdur Y Kristinsson
  • Ulf-Henrik Mellqvist
  • Cecilie Blimark
  • Ingemar Turesson
Enheter & grupper
Externa organisationer
  • Skåne University Hospital
  • Karolinska Institute
  • Regional Cancer Center Western Sweden
  • Umeå University
  • Linköping University Hospital
  • Örebro University Hospital
  • Uppsala University Hospital
  • South Elfsborg Hospital
  • Sahlgrenska University Hospital
Forskningsområden

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Hematologi

Nyckelord

Originalspråkengelska
Sidor (från-till)216-222
TidskriftEuropean Journal of Haematology
Volym99
Utgivningsnummer3
StatusPublished - 2017 sep
PublikationskategoriForskning
Peer review utfördJa