IGLV3-21R110 mutation has prognostic value in patients with treatment-naive chronic lymphocytic leukemia

Chronic lymphocytic leukemia (CLL) has high biological and clinical heterogeneity. A few prognostic factors are used in clinical practice, including immunoglobulin heavy-chain variable (IGHV) gene somatic hypermutation (SHM) status, chromosome aberrations, and gene mutations, which remain insufficie...

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Autores: Syrykh, Charlotte, Pons-Brun, Berta, Russiñol, Núria, Playa-Albinyana, Heribert, Baumann, Tycho, Duran Ferrer, Martí, Kulis, Marta, Carbó-Meix, Anna, Mozas, Pablo, Alcoceba, Miguel, González, Marcos, Navarro-Bailón, Almudena, Colado, Enrique, Payer, Ángel R, Terol, María J, Lu, Junyan, Knisbacher, BBinyamin A, Hahn, Cynthia K, Ruiz-Gaspà, Silvia, Enjuanes, Anna, Wu, Catherine J, Getz, Gad, Zenz, Thorsten, López Guillermo, Armando, Martín-Subero, José Ignacio, Colomer Pujol, Dolors, Delgado González, Julio, Campo Güerri, Elias, Nadeu Prat, Ferran, Aymerich Gregorio, Marta
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2023
País:España
Institución:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositorio:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:2445/223819
Acceso en línea:https://hdl.handle.net/2445/223819
Access Level:acceso abierto
Palabra clave:Pronòstic mèdic
Leucèmia
Prognosis
Leukemia
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spelling IGLV3-21R110 mutation has prognostic value in patients with treatment-naive chronic lymphocytic leukemiaSyrykh, CharlottePons-Brun, BertaRussiñol, NúriaPlaya-Albinyana, HeribertBaumann, TychoDuran Ferrer, MartíKulis, MartaCarbó-Meix, AnnaMozas, PabloAlcoceba, MiguelGonzález, MarcosNavarro-Bailón, AlmudenaColado, EnriquePayer, Ángel RTerol, María JLu, JunyanKnisbacher, BBinyamin AHahn, Cynthia KRuiz-Gaspà, SilviaEnjuanes, AnnaWu, Catherine JGetz, GadZenz, ThorstenLópez Guillermo, ArmandoMartín-Subero, José IgnacioColomer Pujol, DolorsDelgado González, JulioCampo Güerri, EliasNadeu Prat, FerranAymerich Gregorio, MartaPronòstic mèdicLeucèmiaPrognosisLeukemiaChronic lymphocytic leukemia (CLL) has high biological and clinical heterogeneity. A few prognostic factors are used in clinical practice, including immunoglobulin heavy-chain variable (IGHV) gene somatic hypermutation (SHM) status, chromosome aberrations, and gene mutations, which remain insufficient for personalized patient management. Recent studies have shown that expression of the immunoglobulin lambda light chain IGLV3-21R110 gene carrying an SHM-derived G>C mutation changing the glycine at position 110 to an arginine (IGLV3-21 R110 ) defines a subset of CLL with an intermediate epigenetic profile and an aggressive clinical course. When occurring on the IGLV3-21*01 or *04 alleles, the R110 mutation allows homotypic B-cell receptor (BCR) interactions, triggering cell-autonomous BCR signaling and/or facilitating T-cell–independent engagement with superantigen. IGLV3-21 has been detected in up to 6.5% of patients with CLL at diagnosis and in up to 25% of patients enrolled in clinical trials. We and others have shown that all CLL cases belonging to aggressive stereotyped subset #2 carried the IGLV3-21 R110 . Nonetheless, approximately half of IGLV3-21 R110 CLL are not classified as stereotyped subset #2 but seem to have a similar clinical outcome, 5,6 suggesting that the conventional stereotyped subset #2 classification might not completely recognize this clinically aggressive subgroup of CLL. In addition, IGLV3-21 R110 seems to have a prognostic value independent of the IGHV gene SHM status and methylation–based epigenetic subtypes. However, further studies in independent cohorts are needed to support its application in clinical practice. The aim of this study was to assess the prognostic value of IGLV3-21 R110 in large and independent population-based cohorts of patients with CLL.American Society of Hematology2025202520232025info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion8 p.application/pdfhttps://hdl.handle.net/2445/223819Articles publicats en revistes (Fonaments Clínics)reponame:Recercat. Dipósit de la Recerca de Catalunyainstname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)InglésReproducció del document publicat a: https://doi.org/10.1182/bloodadvances.2023010132Blood Advances, 2023, vol. 7, num.23, p. 7384-7391https://doi.org/10.1182/bloodadvances.2023010132(c) American Society of Hematology, 2023info:eu-repo/semantics/openAccessoai:recercat.cat:2445/2238192026-05-29T05:05:01Z
dc.title.none.fl_str_mv IGLV3-21R110 mutation has prognostic value in patients with treatment-naive chronic lymphocytic leukemia
title IGLV3-21R110 mutation has prognostic value in patients with treatment-naive chronic lymphocytic leukemia
spellingShingle IGLV3-21R110 mutation has prognostic value in patients with treatment-naive chronic lymphocytic leukemia
Syrykh, Charlotte
Pronòstic mèdic
Leucèmia
Prognosis
Leukemia
title_short IGLV3-21R110 mutation has prognostic value in patients with treatment-naive chronic lymphocytic leukemia
title_full IGLV3-21R110 mutation has prognostic value in patients with treatment-naive chronic lymphocytic leukemia
title_fullStr IGLV3-21R110 mutation has prognostic value in patients with treatment-naive chronic lymphocytic leukemia
title_full_unstemmed IGLV3-21R110 mutation has prognostic value in patients with treatment-naive chronic lymphocytic leukemia
title_sort IGLV3-21R110 mutation has prognostic value in patients with treatment-naive chronic lymphocytic leukemia
dc.creator.none.fl_str_mv Syrykh, Charlotte
Pons-Brun, Berta
Russiñol, Núria
Playa-Albinyana, Heribert
Baumann, Tycho
Duran Ferrer, Martí
Kulis, Marta
Carbó-Meix, Anna
Mozas, Pablo
Alcoceba, Miguel
González, Marcos
Navarro-Bailón, Almudena
Colado, Enrique
Payer, Ángel R
Terol, María J
Lu, Junyan
Knisbacher, BBinyamin A
Hahn, Cynthia K
Ruiz-Gaspà, Silvia
Enjuanes, Anna
Wu, Catherine J
Getz, Gad
Zenz, Thorsten
López Guillermo, Armando
Martín-Subero, José Ignacio
Colomer Pujol, Dolors
Delgado González, Julio
Campo Güerri, Elias
Nadeu Prat, Ferran
Aymerich Gregorio, Marta
author Syrykh, Charlotte
author_facet Syrykh, Charlotte
Pons-Brun, Berta
Russiñol, Núria
Playa-Albinyana, Heribert
Baumann, Tycho
Duran Ferrer, Martí
Kulis, Marta
Carbó-Meix, Anna
Mozas, Pablo
Alcoceba, Miguel
González, Marcos
Navarro-Bailón, Almudena
Colado, Enrique
Payer, Ángel R
Terol, María J
Lu, Junyan
Knisbacher, BBinyamin A
Hahn, Cynthia K
Ruiz-Gaspà, Silvia
Enjuanes, Anna
Wu, Catherine J
Getz, Gad
Zenz, Thorsten
López Guillermo, Armando
Martín-Subero, José Ignacio
Colomer Pujol, Dolors
Delgado González, Julio
Campo Güerri, Elias
Nadeu Prat, Ferran
Aymerich Gregorio, Marta
author_role author
author2 Pons-Brun, Berta
Russiñol, Núria
Playa-Albinyana, Heribert
Baumann, Tycho
Duran Ferrer, Martí
Kulis, Marta
Carbó-Meix, Anna
Mozas, Pablo
Alcoceba, Miguel
González, Marcos
Navarro-Bailón, Almudena
Colado, Enrique
Payer, Ángel R
Terol, María J
Lu, Junyan
Knisbacher, BBinyamin A
Hahn, Cynthia K
Ruiz-Gaspà, Silvia
Enjuanes, Anna
Wu, Catherine J
Getz, Gad
Zenz, Thorsten
López Guillermo, Armando
Martín-Subero, José Ignacio
Colomer Pujol, Dolors
Delgado González, Julio
Campo Güerri, Elias
Nadeu Prat, Ferran
Aymerich Gregorio, Marta
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Pronòstic mèdic
Leucèmia
Prognosis
Leukemia
topic Pronòstic mèdic
Leucèmia
Prognosis
Leukemia
description Chronic lymphocytic leukemia (CLL) has high biological and clinical heterogeneity. A few prognostic factors are used in clinical practice, including immunoglobulin heavy-chain variable (IGHV) gene somatic hypermutation (SHM) status, chromosome aberrations, and gene mutations, which remain insufficient for personalized patient management. Recent studies have shown that expression of the immunoglobulin lambda light chain IGLV3-21R110 gene carrying an SHM-derived G>C mutation changing the glycine at position 110 to an arginine (IGLV3-21 R110 ) defines a subset of CLL with an intermediate epigenetic profile and an aggressive clinical course. When occurring on the IGLV3-21*01 or *04 alleles, the R110 mutation allows homotypic B-cell receptor (BCR) interactions, triggering cell-autonomous BCR signaling and/or facilitating T-cell–independent engagement with superantigen. IGLV3-21 has been detected in up to 6.5% of patients with CLL at diagnosis and in up to 25% of patients enrolled in clinical trials. We and others have shown that all CLL cases belonging to aggressive stereotyped subset #2 carried the IGLV3-21 R110 . Nonetheless, approximately half of IGLV3-21 R110 CLL are not classified as stereotyped subset #2 but seem to have a similar clinical outcome, 5,6 suggesting that the conventional stereotyped subset #2 classification might not completely recognize this clinically aggressive subgroup of CLL. In addition, IGLV3-21 R110 seems to have a prognostic value independent of the IGHV gene SHM status and methylation–based epigenetic subtypes. However, further studies in independent cohorts are needed to support its application in clinical practice. The aim of this study was to assess the prognostic value of IGLV3-21 R110 in large and independent population-based cohorts of patients with CLL.
publishDate 2023
dc.date.none.fl_str_mv 2023
2025
2025
2025
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/2445/223819
url https://hdl.handle.net/2445/223819
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Reproducció del document publicat a: https://doi.org/10.1182/bloodadvances.2023010132
Blood Advances, 2023, vol. 7, num.23, p. 7384-7391
https://doi.org/10.1182/bloodadvances.2023010132
dc.rights.none.fl_str_mv (c) American Society of Hematology, 2023
info:eu-repo/semantics/openAccess
rights_invalid_str_mv (c) American Society of Hematology, 2023
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv 8 p.
application/pdf
dc.publisher.none.fl_str_mv American Society of Hematology
publisher.none.fl_str_mv American Society of Hematology
dc.source.none.fl_str_mv Articles publicats en revistes (Fonaments Clínics)
reponame:Recercat. Dipósit de la Recerca de Catalunya
instname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
instname_str Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
reponame_str Recercat. Dipósit de la Recerca de Catalunya
collection Recercat. Dipósit de la Recerca de Catalunya
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,811543

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