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  • 1.
    Nahi, Hareth
    et al.
    Karolinska Institute, Sweden.
    Genell, Anna
    Regional Cancer Centre West, Sweden.
    Walinder, Goran
    Karolinska Institute, Sweden.
    Uttervall, Katarina
    Karolinska Institute, Sweden.
    Juliusson, Gunnar
    Lund University, Sweden.
    Karin, Forsberg
    Umeå University Hospital, Sweden.
    Hansson, Markus
    Lund University, Sweden.
    Svensson, Ronald
    Linköping University, Department of Medical and Health Sciences. Linköping University, Faculty of Medicine and Health Sciences. Region Östergötland, Center for Surgery, Orthopaedics and Cancer Treatment, Department of Haematology.
    Linder, Olle
    Örebro University Hospital, Sweden.
    Carlson, Kristina
    Uppsala University Hospital, Sweden.
    Bjorkstrand, Bo
    Karolinska Institute, Sweden.
    Kristinsson, Sigurdur Y.
    Karolinska Institute, Sweden.
    Henrik Mellqvist, Ulf
    South Elvsborg Hospital, Sweden.
    Blimark, Cecilie
    Sahlgrens University Hospital, Sweden.
    Turesson, Ingemar
    Skåne University Hospital, Sweden.
    Incidence, characteristics, and outcome of solitary plasmacytoma and plasma cell leukemia. Population-based data from the Swedish Myeloma Register2017In: European Journal of Haematology, ISSN 0902-4441, E-ISSN 1600-0609, Vol. 99, no 3, p. 216-222Article in journal (Refereed)
    Abstract [en]

    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 2years, 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 11months, 0% at 5years). 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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