TY - JOUR
T1 - Outcome of paraosseous extra-medullary disease in newly diagnosed multiple myeloma patients treated with new drugs
AU - Montefusco, Vittorio
AU - Gay, Francesca
AU - Spada, Stefano
AU - De Paoli, Lorenzo
AU - Di Raimondo, Francesco
AU - Ribolla, Rossella
AU - Musolino, Caterina
AU - Patriarca, Francesca
AU - Musto, Pellegrino
AU - Galieni, Pietro
AU - Ballanti, Stelvio
AU - Nozzoli, Chiara
AU - Cascavilla, Nicola
AU - Ben-Yehuda, Dina
AU - Nagler, Arnon
AU - Hajek, Roman
AU - Offidani, Massimo
AU - Liberati, Anna Marina
AU - Sonneveld, Pieter
AU - Cavo, Michele
AU - Corradini, Paolo
AU - Boccadoro, Mario
N1 - Copyright © 2019, Ferrata Storti Foundation.
PY - 2019/6/20
Y1 - 2019/6/20
N2 - Extramedullary disease is relatively frequent in multiple myeloma, but our knowledge on the subject is limited and mainly relies on small case series or single center experiences. Little is known regarding the role of new drugs in this setting. We performed a meta-analysis of 8 trials focused on the description of extramedullary disease characteristics, clinical outcome, and response to new drugs. A total of 2332 newly diagnosed myeloma patients have been included, 267 (11.4%) had extramedullary disease, defined as paraosseous in 243 (10.4%), extramedullary plasmocytoma in 12 (0.5%), and not classified in 12 (0.5%) patients. Median progression-free survival was 25.3 months and 25.2 in extramedullary disease and non-extramedullary disease patients, respectively. In multivariate analysis the presence of extramedullary disease did not impact on progression-free survival (hazard ratio 1.15, p=0.06), while other known prognostic factors retained their significance. Patients treated with immunomodulatory drugs, mainly lenalidomide, or proteasome inhibitors had similar progression-free survival and progression-free survival-2 regardless of extramedullary disease presence. Median overall survival was 63.5 months and 79.9 months (p=0.01) in extramedullary and non-extramedullary disease patients, respectively, and in multivariate analysis the presence of extramedullary disease was associated with a reduced overall survival (hazard ratio 1.41, p<0.001), in line with other prognostic factors. With the limits of the low sensitivity imaging techniques used in this trial, that lead to an underestimation of extramedullary disease, we conclude that in patients treated with new drugs the detrimental effect of extramedullary disease at diagnosis is limited, that lenalidomide is effective as are proteasome inhibitors, and that these patients tend to acquire a more aggressive disease in later stages. (EUDRACT2005-004714-32, NCT01063179 NCT00551928, NCT01091831, NCT01093196, NCT01190787, NCT01346787, NCT01857115).
AB - Extramedullary disease is relatively frequent in multiple myeloma, but our knowledge on the subject is limited and mainly relies on small case series or single center experiences. Little is known regarding the role of new drugs in this setting. We performed a meta-analysis of 8 trials focused on the description of extramedullary disease characteristics, clinical outcome, and response to new drugs. A total of 2332 newly diagnosed myeloma patients have been included, 267 (11.4%) had extramedullary disease, defined as paraosseous in 243 (10.4%), extramedullary plasmocytoma in 12 (0.5%), and not classified in 12 (0.5%) patients. Median progression-free survival was 25.3 months and 25.2 in extramedullary disease and non-extramedullary disease patients, respectively. In multivariate analysis the presence of extramedullary disease did not impact on progression-free survival (hazard ratio 1.15, p=0.06), while other known prognostic factors retained their significance. Patients treated with immunomodulatory drugs, mainly lenalidomide, or proteasome inhibitors had similar progression-free survival and progression-free survival-2 regardless of extramedullary disease presence. Median overall survival was 63.5 months and 79.9 months (p=0.01) in extramedullary and non-extramedullary disease patients, respectively, and in multivariate analysis the presence of extramedullary disease was associated with a reduced overall survival (hazard ratio 1.41, p<0.001), in line with other prognostic factors. With the limits of the low sensitivity imaging techniques used in this trial, that lead to an underestimation of extramedullary disease, we conclude that in patients treated with new drugs the detrimental effect of extramedullary disease at diagnosis is limited, that lenalidomide is effective as are proteasome inhibitors, and that these patients tend to acquire a more aggressive disease in later stages. (EUDRACT2005-004714-32, NCT01063179 NCT00551928, NCT01091831, NCT01093196, NCT01190787, NCT01346787, NCT01857115).
U2 - 10.3324/haematol.2019.219139
DO - 10.3324/haematol.2019.219139
M3 - Article
C2 - 31221778
JO - Haematologica
JF - Haematologica
SN - 0390-6078
ER -