Incidence, characteristics, and outcome of solitary plasmacytoma and plasma cell leukemia. Population-based data from the Swedish Myeloma Register
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Authors
Nahi, HarethGenell, 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
Issue Date
2017-09
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Incidence, characteristics, and outcome of solitary plasmacytoma and plasma cell leukemia. Population-based data from the Swedish Myeloma Register 2017, 99 (3):216 European Journal of HaematologyAbstract
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.Description
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http://doi.wiley.com/10.1111/ejh.12907Rights
Archived with thanks to European Journal of Haematologyae974a485f413a2113503eed53cd6c53
10.1111/ejh.12907
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