Aberrant T-cell exhaustion in severe combined immunodeficiency survivors with poor T-cell reconstitution after transplantation.
Conditioning chemotherapy
T-cell exhaustion
hematopoietic cell transplantation (HCT)
immune reconstitution
severe combined immunodeficiency (SCID)
Journal
The Journal of allergy and clinical immunology
ISSN: 1097-6825
Titre abrégé: J Allergy Clin Immunol
Pays: United States
ID NLM: 1275002
Informations de publication
Date de publication:
01 2023
01 2023
Historique:
received:
11
04
2022
revised:
08
08
2022
accepted:
10
08
2022
pmc-release:
01
01
2024
pubmed:
21
8
2022
medline:
11
1
2023
entrez:
20
8
2022
Statut:
ppublish
Résumé
Severe combined immunodeficiency (SCID) comprises rare inherited disorders of immunity that require definitive treatment through hematopoietic cell transplantation (HCT) or gene therapy for survival. Despite successes of allogeneic HCT, many SCID patients experience incomplete immune reconstitution, persistent T-cell lymphopenia, and poor long-term outcomes. We hypothesized that CD4 We analyzed markers of exhaustion in blood samples from 61 SCID patients at a median of 10.4 years after HCT. Compared to post-HCT SCID patients with normal CD4 Recipients of unconditioned HCT for SCID may develop late post-HCT T-cell exhaustion as a result of diminished production of T-lineage cells. Elevated expression of inhibitory receptors on their T cells may be a biomarker of poor long-term T-cell reconstitution.
Sections du résumé
BACKGROUND
Severe combined immunodeficiency (SCID) comprises rare inherited disorders of immunity that require definitive treatment through hematopoietic cell transplantation (HCT) or gene therapy for survival. Despite successes of allogeneic HCT, many SCID patients experience incomplete immune reconstitution, persistent T-cell lymphopenia, and poor long-term outcomes.
OBJECTIVE
We hypothesized that CD4
METHODS
We analyzed markers of exhaustion in blood samples from 61 SCID patients at a median of 10.4 years after HCT.
RESULTS
Compared to post-HCT SCID patients with normal CD4
CONCLUSIONS
Recipients of unconditioned HCT for SCID may develop late post-HCT T-cell exhaustion as a result of diminished production of T-lineage cells. Elevated expression of inhibitory receptors on their T cells may be a biomarker of poor long-term T-cell reconstitution.
Identifiants
pubmed: 35987350
pii: S0091-6749(22)01057-0
doi: 10.1016/j.jaci.2022.08.004
pmc: PMC9924130
mid: NIHMS1831997
pii:
doi:
Substances chimiques
Receptors, Antigen, T-Cell
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, Non-P.H.S.
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
260-271Subventions
Organisme : NIAID NIH HHS
ID : R13 AI094943
Pays : United States
Organisme : CIHR
ID : PJYT-175209
Pays : Canada
Organisme : NCI NIH HHS
ID : U24 CA076518
Pays : United States
Organisme : NIAID NIH HHS
ID : U54 AI082973
Pays : United States
Organisme : NCI NIH HHS
ID : P30 CA008748
Pays : United States
Organisme : NHLBI NIH HHS
ID : U01 HL069294
Pays : United States
Organisme : NINDS NIH HHS
ID : U54 NS064808
Pays : United States
Organisme : NCATS NIH HHS
ID : U01 TR001263
Pays : United States
Organisme : NHLBI NIH HHS
ID : U10 HL069254
Pays : United States
Organisme : CIHR
ID : MOP-130469
Pays : Canada
Informations de copyright
Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.
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