T-cell immune profile in blood of systemic mastocytosis: Association with disease features

[Background]: Systemic mastocytosis (SM) is a heterogeneous disease characterized by an expansion of KIT-mutated mast cells (MC). KIT-mutated MC display activated features and release MC mediators that might act on the tumour microenvironment and other immune cells. Here, we investigated the distrib...

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Autores: Pérez-Pons, Alba, Teodosio, Cristina, Jara-Acevedo, Maria, Henriques, Ana, Navarro-Navarro, Paula, García-Montero, Andrés, Álvarez-Twose, Iván, Lécrevisse, Quentin, Fluxá, Rafael, Sánchez-Muñoz, Laura, Caldas, Carolina, Pozo, Julio, Martín, Silvia, Contreras-Sanfeliciano, Teresa, Pedreira, C. E., Botafogo, Vitor, González-López, Óscar, Mayado, Andrea, Orfao, Alberto
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2024
País:España
Institución:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/382038
Acceso en línea:http://hdl.handle.net/10261/382038
Access Level:acceso abierto
Palabra clave:Cytotoxic T cells
Helper T cells
Lymphocytes
Mast cells
Systemic mastocytosis
id ES_2bcb5fdb83bbf9b0fc8671d9fe5ea6b5
oai_identifier_str oai:digital.csic.es:10261/382038
network_acronym_str ES
network_name_str España
repository_id_str
dc.title.none.fl_str_mv T-cell immune profile in blood of systemic mastocytosis: Association with disease features
title T-cell immune profile in blood of systemic mastocytosis: Association with disease features
spellingShingle T-cell immune profile in blood of systemic mastocytosis: Association with disease features
Pérez-Pons, Alba
Cytotoxic T cells
Helper T cells
Lymphocytes
Mast cells
Systemic mastocytosis
title_short T-cell immune profile in blood of systemic mastocytosis: Association with disease features
title_full T-cell immune profile in blood of systemic mastocytosis: Association with disease features
title_fullStr T-cell immune profile in blood of systemic mastocytosis: Association with disease features
title_full_unstemmed T-cell immune profile in blood of systemic mastocytosis: Association with disease features
title_sort T-cell immune profile in blood of systemic mastocytosis: Association with disease features
dc.creator.none.fl_str_mv Pérez-Pons, Alba
Teodosio, Cristina
Jara-Acevedo, Maria
Henriques, Ana
Navarro-Navarro, Paula
García-Montero, Andrés
Álvarez-Twose, Iván
Lécrevisse, Quentin
Fluxá, Rafael
Sánchez-Muñoz, Laura
Caldas, Carolina
Pozo, Julio
Martín, Silvia
Contreras-Sanfeliciano, Teresa
Pedreira, C. E.
Botafogo, Vitor
González-López, Óscar
Mayado, Andrea
Orfao, Alberto
author Pérez-Pons, Alba
author_facet Pérez-Pons, Alba
Teodosio, Cristina
Jara-Acevedo, Maria
Henriques, Ana
Navarro-Navarro, Paula
García-Montero, Andrés
Álvarez-Twose, Iván
Lécrevisse, Quentin
Fluxá, Rafael
Sánchez-Muñoz, Laura
Caldas, Carolina
Pozo, Julio
Martín, Silvia
Contreras-Sanfeliciano, Teresa
Pedreira, C. E.
Botafogo, Vitor
González-López, Óscar
Mayado, Andrea
Orfao, Alberto
author_role author
author2 Teodosio, Cristina
Jara-Acevedo, Maria
Henriques, Ana
Navarro-Navarro, Paula
García-Montero, Andrés
Álvarez-Twose, Iván
Lécrevisse, Quentin
Fluxá, Rafael
Sánchez-Muñoz, Laura
Caldas, Carolina
Pozo, Julio
Martín, Silvia
Contreras-Sanfeliciano, Teresa
Pedreira, C. E.
Botafogo, Vitor
González-López, Óscar
Mayado, Andrea
Orfao, Alberto
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Instituto de Salud Carlos III
Centro de Investigación Biomédica en Red Cáncer (España)
European Commission
Ministerio de Ciencia, Innovación y Universidades (España)
Junta de Castilla y León
Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]
dc.subject.none.fl_str_mv Cytotoxic T cells
Helper T cells
Lymphocytes
Mast cells
Systemic mastocytosis
topic Cytotoxic T cells
Helper T cells
Lymphocytes
Mast cells
Systemic mastocytosis
description [Background]: Systemic mastocytosis (SM) is a heterogeneous disease characterized by an expansion of KIT-mutated mast cells (MC). KIT-mutated MC display activated features and release MC mediators that might act on the tumour microenvironment and other immune cells. Here, we investigated the distribution of lymphocyte subsets in blood of patients with distinct subtypes of SM and determined its association with other disease features.
publishDate 2024
dc.date.none.fl_str_mv 2024
2025
2025
dc.type.none.fl_str_mv info:eu-repo/semantics/article
http://purl.org/coar/resource_type/c_6501
Publisher's version
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/10261/382038
url http://hdl.handle.net/10261/382038
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv #PLACEHOLDER_PARENT_METADATA_VALUE#
info:eu-repo/grantAgreement/EC/H2020/101034371
The underlying dataset has been published as supplementary material of the article in the publisher platform at DOI https://doi.org/10.1111/all.16043
https://doi.org/10.1111/all.16043
Sí
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv John Wiley & Sons
publisher.none.fl_str_mv John Wiley & Sons
dc.source.none.fl_str_mv reponame:DIGITAL.CSIC. Repositorio Institucional del CSIC
instname:Consejo Superior de Investigaciones Científicas (CSIC)
instname_str Consejo Superior de Investigaciones Científicas (CSIC)
reponame_str DIGITAL.CSIC. Repositorio Institucional del CSIC
collection DIGITAL.CSIC. Repositorio Institucional del CSIC
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869405181126377472
spelling T-cell immune profile in blood of systemic mastocytosis: Association with disease featuresPérez-Pons, AlbaTeodosio, CristinaJara-Acevedo, MariaHenriques, AnaNavarro-Navarro, PaulaGarcía-Montero, AndrésÁlvarez-Twose, IvánLécrevisse, QuentinFluxá, RafaelSánchez-Muñoz, LauraCaldas, CarolinaPozo, JulioMartín, SilviaContreras-Sanfeliciano, TeresaPedreira, C. E.Botafogo, VitorGonzález-López, ÓscarMayado, AndreaOrfao, AlbertoCytotoxic T cellsHelper T cellsLymphocytesMast cellsSystemic mastocytosis[Background]: Systemic mastocytosis (SM) is a heterogeneous disease characterized by an expansion of KIT-mutated mast cells (MC). KIT-mutated MC display activated features and release MC mediators that might act on the tumour microenvironment and other immune cells. Here, we investigated the distribution of lymphocyte subsets in blood of patients with distinct subtypes of SM and determined its association with other disease features.[Methods]: We studied the distribution of TCD4+ and TCD4− cytotoxic cells and their subsets, as well as total NK- and B cells, in blood of 115 SM patients—38 bone marrow mastocytosis (BMM), 67 indolent SM (ISM), 10 aggressive SM (ASM)- and 83 age-matched healthy donors (HD), using spectral flow cytometry and the EuroFlow Immunomonitoring panel, and correlated it with multilineage KITD816V, the alpha-tryptasemia genotype (HαT) and the clinical manifestations of the disease.[Results]: SM patients showed decreased counts (vs. HD) of TCD4− cytotoxic cells, NK cells and several functional subsets of TCD4+ cells (total Th1, Th2-effector memory, Th22-terminal effector and Th1-like Tregs), together with increased T-follicular-helper and Th1/Th17-like Treg counts, associated with different immune profiles per diagnostic subtype of SM, in multilineal versus MC-restricted KITD816V and in cases with a HαT+ versus HαT− genotype. Unique immune profiles were found among BMM and ISM patients with MC-restricted KITD816V who displayed HαT, anaphylaxis, hymenoptera venom allergy, bone disease, pruritus, flushing and GI symptoms.[Conclusion]: Our results reveal altered T- and NK-cell immune profiles in blood of SM, which vary per disease subtype, the pattern of involvement of haematopoiesis by KITD816V, the HαT genotype and specific clinical manifestations of the disease.This study has been supported by the following grants: grant number PI19/01166 and PI22/01657 from Instituto de Salud Carlos III (ISCIII; Madrid, Spain); grant number CB16/12/00400 and from the Biomedical Research Networking Center Consortium [CIBERONC] programme (ISCIII, Madrid, Spain); co-funded by the ‘European Union’ (EQC2019-005419-P), within the Subprograma Estatal de Infraestructuras de Investigación y Equipamiento Científico Técnico de 2019 del Ministerio de Ciencia, Innovación y Universidades. APP was supported by a grant from the Government of Castilla y León (Orden EDU/556/2019; Valladolid, Spain) co-funded by the ‘European Regional Development Fund’ (FEDER BDNS, Identif.: 422058). CT is the recipient of an USAL4EXCELLENCE fellowship, co-funded by the European Union's Horizon 2020 research and innovation programme under the Marie Skłodowska-Curie grant agreement No. 101034371 and the Junta de Castilla y León. PNN was supported by a grant from the Government of Castilla y León (Orden EDU/875/2021: Valladolid, Spain) co-funded by the European Social Fund (FEDER BDNS, Identif.: 540787). OGL was supported by a grant from the Instituto de Salud Carlos III (ISCIII) (PFIS: FI20/00116-AES 2017–2020, BOE 28/03/2019-Orden CNU/354/2019) co-funded by The European Union (FSE+). AM was supported by the Biomedical Research Networking Center Consortium on cancer (CIBERONC) (contract number PRF-2869).Peer reviewedJohn Wiley & SonsInstituto de Salud Carlos IIICentro de Investigación Biomédica en Red Cáncer (España)European CommissionMinisterio de Ciencia, Innovación y Universidades (España)Junta de Castilla y LeónConsejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]202520252024info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_6501Publisher's versioninfo:eu-repo/semantics/publishedVersionapplication/pdfhttp://hdl.handle.net/10261/382038reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Inglés#PLACEHOLDER_PARENT_METADATA_VALUE#info:eu-repo/grantAgreement/EC/H2020/101034371The underlying dataset has been published as supplementary material of the article in the publisher platform at DOI https://doi.org/10.1111/all.16043https://doi.org/10.1111/all.16043Síinfo:eu-repo/semantics/openAccessoai:digital.csic.es:10261/3820382026-05-22T06:33:51Z
score 15,811543

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