Pubblicazioni

Autori:

Bonfiglio, Ferdinando; Bruscaggin, Alessio; Guidetti, Francesca; Terzi di Bergamo, Lodovico; Faderl, Martin; Spina, Valeria; Condoluci, Adalgisa; Bonomini, Luisella; Forestieri, Gabriela; Koch, Ricardo; Piffaretti, Deborah; Pini, Katia; Pirosa, Maria Cristina; Cittone, Micol Giulia; Arribas, Alberto; Lucioni, Marco; Ghilardi, Guido; Wu, Wei; Arcaini, Luca; Baptista, Maria Joao; Bastidas, Gabriela; Bea, Silvia; Boldorini, Renzo; Broccoli, Alessandro; Buehler, Marco Matteo; Canzonieri, Vincenzo; Cascione, Luciano; Ceriani, Luca; Cogliatti, Sergio; Corradini, Paolo; Derenzini, Enrico; Devizzi, Liliana; Dietrich, Sascha; Elia, Angela Rita; Facchetti, Fabio; Gaidano, Gianluca; Garcia, Juan Fernando; Gerber, Bernhard; Ghia, Paolo; Gomes da Silva, Maria; Gritti, Giuseppe; Guidetti, Anna; Hitz, Felicitas; Inghirami, Giorgio; Ladetto, Marco; Lopez-Guillermo, Armando; Lucchini, Elisa; Maiorana, Antonino; Marasca, Roberto; Matutes, Estella; Meignin, Veronique; Merli, Michele; Moccia, Alden; Mollejo, Manuela; Montalban, Carlos; Novak, Urban; Oscier, David Graham; Passamonti, Francesco; Piazza, Francesco; Pizzolitto, Stefano; Rambaldi, Alessandro; Sabattini, Elena; Salles, Gilles; Santambrogio, Elisa; Scarfò, Lydia; Stathis, Anastasios; Stüssi, Georg; Geyer, Julia T; Tapia, Gustavo; Tarella, Corrado; Thieblemont, Catherine; Tousseyn, Thomas; Tucci, Alessandra; Vanini, Giorgio; Visco, Carlo; Vitolo, Umberto; Walewska, Renata; Zaja, Francesco; Zenz, Thorsten; Zinzani, Pier Luigi; Khiabanian, Hossein; Calcinotto, Arianna; Bertoni, Francesco; Bhagat, Govind; Campo, Elias; De Leval, Laurence; Dirnhofer, Stefan; Pileri, Stefano A; Piris, Miguel A; Traverse-Glehen, Alexandra; Tzankov, Alexander; Paulli, Marco; Ponzoni, Maurilio; Mazzucchelli, Luca; Cavalli, Franco; Zucca, Emanuele; Rossi, Davide

Titolo:

Genetic and phenotypic attributes of splenic marginal zone lymphoma

Anno:

2022

Tipologia prodotto:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Lingua:

Inglese

Referee:

No

Nome rivista:

Blood

ISSN Rivista:

0006-4971

N° Volume:

139

Numero o Fascicolo:

5

Editore:

American Society of Hematology

Intervallo pagine:

732-747

Parole chiave:

Aged; Animals; Chromosome Aberrations; Female; Humans; Immunophenotyping; Lymphoma, B-Cell, Marginal Zone; Male; Mice; Middle Aged; Multigene Family; Mutation; Spleen; Splenic Neoplasms; Transcriptome; Tumor Microenvironment

Breve descrizione dei contenuti:

: Splenic marginal zone B-cell lymphoma (SMZL) is a heterogeneous clinico-biological entity. The clinical course is variable, multiple genes are mutated with no unifying mechanism, and essential regulatory pathways and surrounding microenvironments are diverse. We sought to clarify the heterogeneity of SMZL by resolving different subgroups and their underlying genomic abnormalities, pathway signatures, and microenvironment compositions to uncover biomarkers and therapeutic vulnerabilities. We studied 303 SMZL spleen samples collected through the IELSG46 multicenter international study (NCT02945319) by using a multiplatform approach. We carried out genetic and phenotypic analyses, defined self-organized signatures, validated the findings in independent primary tumor metadata and in genetically modified mouse models, and determined correlations with outcome data. We identified 2 prominent genetic clusters in SMZL, termed NNK (58% of cases, harboring NF-κB, NOTCH, and KLF2 modules) and DMT (32% of cases, with DNA-damage response, MAPK, and TLR modules). Genetic aberrations in multiple genes as well as cytogenetic and immunogenetic features distinguished NNK- from DMT-SMZLs. These genetic clusters not only have distinct underpinning biology, as judged by differences in gene-expression signatures, but also different outcomes, with inferior survival in NNK-SMZLs. Digital cytometry and in situ profiling segregated 2 basic types of SMZL immune microenvironments termed immune-suppressive SMZL (50% of cases, associated with inflammatory cells and immune checkpoint activation) and immune-silent SMZL (50% of cases, associated with an immune-excluded phenotype) with distinct mutational and clinical connotations. In summary, we propose a nosology of SMZL that can implement its classification and also aid in the development of rationally targeted treatments.

Id prodotto:

126832

Handle IRIS:

11562/1064983

ultima modifica:

23 gennaio 2025

Citazione bibliografica:

Bonfiglio, Ferdinando; Bruscaggin, Alessio; Guidetti, Francesca; Terzi di Bergamo, Lodovico; Faderl, Martin; Spina, Valeria; Condoluci, Adalgisa; Bonomini, Luisella; Forestieri, Gabriela; Koch, Ricardo; Piffaretti, Deborah; Pini, Katia; Pirosa, Maria Cristina; Cittone, Micol Giulia; Arribas, Alberto; Lucioni, Marco; Ghilardi, Guido; Wu, Wei; Arcaini, Luca; Baptista, Maria Joao; Bastidas, Gabriela; Bea, Silvia; Boldorini, Renzo; Broccoli, Alessandro; Buehler, Marco Matteo; Canzonieri, Vincenzo; Cascione, Luciano; Ceriani, Luca; Cogliatti, Sergio; Corradini, Paolo; Derenzini, Enrico; Devizzi, Liliana; Dietrich, Sascha; Elia, Angela Rita; Facchetti, Fabio; Gaidano, Gianluca; Garcia, Juan Fernando; Gerber, Bernhard; Ghia, Paolo; Gomes da Silva, Maria; Gritti, Giuseppe; Guidetti, Anna; Hitz, Felicitas; Inghirami, Giorgio; Ladetto, Marco; Lopez-Guillermo, Armando; Lucchini, Elisa; Maiorana, Antonino; Marasca, Roberto; Matutes, Estella; Meignin, Veronique; Merli, Michele; Moccia, Alden; Mollejo, Manuela; Montalban, Carlos; Novak, Urban; Oscier, David Graham; Passamonti, Francesco; Piazza, Francesco; Pizzolitto, Stefano; Rambaldi, Alessandro; Sabattini, Elena; Salles, Gilles; Santambrogio, Elisa; Scarfò, Lydia; Stathis, Anastasios; Stüssi, Georg; Geyer, Julia T; Tapia, Gustavo; Tarella, Corrado; Thieblemont, Catherine; Tousseyn, Thomas; Tucci, Alessandra; Vanini, Giorgio; Visco, Carlo; Vitolo, Umberto; Walewska, Renata; Zaja, Francesco; Zenz, Thorsten; Zinzani, Pier Luigi; Khiabanian, Hossein; Calcinotto, Arianna; Bertoni, Francesco; Bhagat, Govind; Campo, Elias; De Leval, Laurence; Dirnhofer, Stefan; Pileri, Stefano A; Piris, Miguel A; Traverse-Glehen, Alexandra; Tzankov, Alexander; Paulli, Marco; Ponzoni, Maurilio; Mazzucchelli, Luca; Cavalli, Franco; Zucca, Emanuele; Rossi, Davide, Genetic and phenotypic attributes of splenic marginal zone lymphoma «Blood» , vol. 139 , n. 5 , 2022 , pp. 732-747

Consulta la scheda completa presente nel repository istituzionale della Ricerca di Ateneo