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Plasma Cell Disorders
A real world multicenter retrospective study on extramedullary disease from Balkan Myeloma Study Group and Barcelona University: analysis of parameters that improve outcome
Ferrata Storti Foundation
Haematologica 2020 Volume 105(1):201-208
Meral Beksac,1 Guldane Cengiz Seval,1 Nicholas Kanellias,2 Daniel Coriu,3 Laura Rosiñol,4 Gulsum Ozet,5 Vesselina Goranova-Marinova,6 Ali Unal,7 Jelena Bila,8 Hayri Ozsan,9 Arben Ivanaj,10 Lejla Ibricevic Balić,11 Efstathios Kastritis,2 Joan Bladé,4 Meletios Athanasios Dimopoulos2
1Department of Hematology, School of Medicine, Ankara University, Ankara, Turkey; 2Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece; 3University of Medicine and Pharmacy "Carol Davila", Fundeni Clinical Institute, Bucharest, Romania; 4Hospital Clinic, IDIBAPS, Barcelona, Spain; 5Clinic of Hematology, Ankara Numune Education and Research Hospital, Ankara, Turkey; 6University Hospital “Sv. Georgi” and Medical University Plovdiv, Plovdiv, Bulgaria; 7Department of Hematology, School of Medicine, Erciyes University, Kayseri, Turkey; 8Faculty of Medicine, University of Belgrade, Belgrade, Serbia; 9Department of Hematology, School of Medicine, Dokuz Eylül University, Izmir, Turkey; 10University of Medicine Tirana, Tirana, Albania and 11Clinical Center of Sarajevo University, Sarajevo, Bosnia and Herzegovina
ABSTRACT
Here, we report the outcome of 226 myeloma patients presenting with extramedullary plasmacytoma or paraosseous involvement in a retro- spective 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 of 38) and in 96 at relapse (84 of 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 emis- sion tomography combined with computed tomography (n=50), or magnetic resonance imaging (n=35). The entire group received a median two lines of treat- ment and autologous stem cell transplantation (44%) following the diagnosis of extramedullary disease. Complete response was higher for paraosseous involve- ment versus 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 (PFS) 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 plas- macytoma at relapse had the worst prognosis with a PFS of 13.6 months and overall survival of 11.4 months. In the multivariate analysis, paraosseous involve- ment, extramedullary disease at diagnosis, International Staging System (ISS-I), and undergoing autologous stem cell transplantation improved overall survival independently. This cohort demonstrated that extramedullary disease benefits from front-line autologous stem cell transplantation and extramedullary plasma- cytoma differs from paraosseous involvement in terms of rate and duration of response, with even worse outcomes when detected at relapse, constituting an unmet clinical need.
Introduction
Multiple myeloma (MM) originates from the proliferation of clonal malignant plasma cells (PC) with a strong interaction with the bone marrow microenviron- ment. Although the disease is considered generally incurable, overall survival (OS)
Correspondence:
MERAL BEKSAC
meral.beksac@medicine.ankara.edu.tr
Received: February 11, 2019. Accepted: July 5, 2019. Pre-published: July 5, 2019.
doi:10.3324/haematol.2019.219295
Check the online version for the most updated information on this article, online supplements, and information on authorship & disclosures: www.haematologica.org/content/105/1/201
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