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Haematologica. 2019 Jul 5. pii: haematol.2019.219295. doi: 10.3324/haematol.2019.219295. [Epub ahead of print]

A real world multicenter retrospective study on extramedullary disease from Balkan Myeloma Study Group and Barcelona University: analysis of parameters that improve outcome.

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Department of Hematology, School of Medicine, Ankara University;
Department of Hematology, School of Medicine, Ankara University.
Department of Clinical Therapeutics, National and Kapodistrian University of Athens.
University of Medicine and Pharmacy.
Hospital Clinic, IDIBAPS.
Clinic of Hematology, Ankara Numune Education and Research Hospital.
University Hospital Sv. Georgi and Medical University Plovdiv.
Department of Hematology, School of Medicine, Erciyes University.
Faculty of Medicine, University of Belgrade.
Department of Hematology, School of Medicine, Dokuz Eylül University.
University of Medicine Tirana.
Clinical Center of Sarajevo University.
Department of Clin ical Therapeutics, National and Kapodostrian University of Athens.


Here, we are reporting the outcome of 226 myeloma patients presenting with extramedullary plasmacytoma or paraosseous involvement in a retrospective study conducted in 19 centers from 11 countries. Extramedullary disease was detected at diagnosis or relapse between January 2010 and November 2017. Extramedullary plasmacytoma and paraosseous involvement were observed in 130 patients at diagnosis (92/38) and in 96 at relapse (84/12). The median time from multiple myeloma diagnosis to the development of extramedullary disease was 25.1 months (range 3.1-106.3 months) in the relapse group (median follow-up: 15 months). Imaging approach for extramedullary disease was computed tomography (n: 133), positron emission tomography combined with computed tomography (n: 50) or magnetic resonance imaging (n: 35). The entire group received median two lines of treatment and autologous stem cell transplantation (44%) following the diagnosis of extramedullary disease. Complete response was higher for paraosseous involvement vs. extramedullary plasmacytoma at diagnosis (34.2 % vs. 19.3 %; p=NS.) and relapse (54.5 % vs. 9 % ; p=0.001). Also paraosseous involvement patients had a better progression free survival when recognized at initial diagnosis of myeloma than at relapse (51.7 vs. 38.9 months). In addition, overall survival was better for paraosseous involvement compared to extramedullary plasmacytoma at diagnosis (not reached vs. 46.5 months). Extramedullary plasmacytoma at relapse had the worst prognosis with a progression free survival (13.6 months) and overall survival (11.4 months). In the multivariate analysis, paraosseous involvement, extramedullary disease at diagnosis, ISS-I and undergoing autologous stem cell transplantation improved overall survival independently. This cohort demonstrated extramedullary disease to benefit from frontline autologous stem cell transplantation. Extramedullary plasmacytoma to differ from paraosseous involvement in terms of rate and duration of response, with even worse outcomes when detected at relapse constituting an unmet need.


Extramedullary disease; Multiple Myeloma; Outcome; Paraosseous; Stem Cell Transplantation

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