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Nahi, Hareth; Genell, Anna; Wålinder, Göran; Uttervall, Katarina; Juliusson, Gunnar; Karin, Forsberg; Hansson, Markus; Svensson, Ronald; Linder, Olle; Carlson, Kristina; Björkstrand, Bo; Kristinsson, Sigurdur Y.; Mellqvist, Ulf Henrik; Blimark, Cecilie; Turesson, Ingemar

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

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  • Media type: E-Article
  • Title: Incidence, characteristics, and outcome of solitary plasmacytoma and plasma cell leukemia. Population‐based data from the Swedish Myeloma Register
  • Contributor: Nahi, Hareth; Genell, Anna; Wålinder, Göran; Uttervall, Katarina; Juliusson, Gunnar; Karin, Forsberg; Hansson, Markus; Svensson, Ronald; Linder, Olle; Carlson, Kristina; Björkstrand, Bo; Kristinsson, Sigurdur Y.; Mellqvist, Ulf Henrik; Blimark, Cecilie; Turesson, Ingemar
  • imprint: Wiley, 2017
  • Published in: European Journal of Haematology
  • Language: English
  • DOI: 10.1111/ejh.12907
  • ISSN: 1600-0609; 0902-4441
  • Origination:
  • Footnote:
  • Description: <jats:title>Abstract</jats:title><jats:p>Solitary plasmacytoma (<jats:styled-content style="fixed-case">SP</jats:styled-content>) and plasma cell leukemia (<jats:styled-content style="fixed-case">PCL</jats:styled-content>) are uncommon (3‐6%) types of plasma cell disease. The risk of progression to symptomatic multiple myeloma (<jats:styled-content style="fixed-case">MM</jats:styled-content>) is probably important for the outcome of <jats:styled-content style="fixed-case">SP</jats:styled-content>. <jats:styled-content style="fixed-case">PCL</jats:styled-content> is rare and has a dismal outcome. In this study, we report on incidence and survival in <jats:styled-content style="fixed-case">PCL</jats:styled-content>/<jats:styled-content style="fixed-case">SP</jats:styled-content>, and progression to <jats:styled-content style="fixed-case">MM</jats:styled-content> in <jats:styled-content style="fixed-case">SP</jats:styled-content>, 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 (<jats:styled-content style="fixed-case">SBP</jats:styled-content>) (<jats:italic>n</jats:italic>=124) and extramedullary plasmacytoma (<jats:styled-content style="fixed-case">EMP</jats:styled-content>) (<jats:italic>n</jats:italic>=67) have better overall survival (<jats:styled-content style="fixed-case">OS</jats:styled-content>) than <jats:styled-content style="fixed-case">MM</jats:styled-content> (<jats:italic>n</jats:italic>=3549). Progression to <jats:styled-content style="fixed-case">MM</jats:styled-content> was higher in <jats:styled-content style="fixed-case">SBP</jats:styled-content> than in <jats:styled-content style="fixed-case">EMP</jats:styled-content> (35% and 7% at 2 years, respectively), but this did not translate into better survival in <jats:styled-content style="fixed-case">EMP</jats:styled-content>. In spite of treatment developments, the <jats:styled-content style="fixed-case">OS</jats:styled-content> of primary <jats:styled-content style="fixed-case">PCL</jats:styled-content> 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 <jats:styled-content style="fixed-case">PCL</jats:styled-content>.</jats:p>