Common variable immunodeficiency–associated endotoxemia promotes early commitment to the T follicular lineage

Although chiefly a B-lymphocyte disorder, several research groups have identified common variable immunodeficiency (CVID) subjects with numeric and/or functional TH cell alterations. The causes, interrelationships, and consequences of CVID-associated CD4+ T-cell derangements to hypogammaglobulinemia...

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Vydáno v:	Journal of allergy and clinical immunology Ročník 144; číslo 6; s. 1660 - 1673
Hlavní autoři:	Le Coz, Carole, Bengsch, Bertram, Khanna, Caroline, Trofa, Melissa, Ohtani, Takuya, Nolan, Brian E., Henrickson, Sarah E., Lambert, Michele P., Kim, Taylor Olmsted, Despotovic, Jenny M., Feldman, Scott, Fadugba, Olajumoke O., Takach, Patricia, Ruffner, Melanie, Jyonouchi, Soma, Heimall, Jennifer, Sullivan, Kathleen E., Wherry, E. John, Romberg, Neil
Médium:	Journal Article
Jazyk:	angličtina
Vydáno:	United States Elsevier Inc 01.12.2019 Elsevier Limited
Témata:	Activin activin A Age Anemia Autoantibodies autoimmune cytopenias CD4 antigen Cell differentiation Cell lineage Chemokines Common variable immunodeficiency Costimulator Cytokines Cytopenia Endotoxemia endotoxin Enrollments Flow cytometry follicular helper T cell Hypogammaglobulinemia Immunoglobulin A Lymphocytes B Lymphocytes T Pathogenesis recent thymic emigrant regulatory T cell Signal transduction Software Thymus time-of-flight cytometry Transcription RTE Common variable immunodeficiency CVID−AIC ITP t-SNE FACS follicular helper T cell CVID time-of-flight cytometry TFH AIC TLR ICOS CSR Treg recent thymic emigrant autoimmune cytopenias CFSE ICOSL regulatory T cell FOXP3 ES CVID+AIC activin A HD cTFH CyTOF PD-1 endotoxin AIHA
ISSN:	0091-6749, 1097-6825, 1097-6825
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Abstract	Although chiefly a B-lymphocyte disorder, several research groups have identified common variable immunodeficiency (CVID) subjects with numeric and/or functional TH cell alterations. The causes, interrelationships, and consequences of CVID-associated CD4+ T-cell derangements to hypogammaglobulinemia, autoantibody production, or both remain unclear. We sought to determine how circulating CD4+ T cells are altered in CVID subjects with autoimmune cytopenias (AICs; CVID+AIC) and the causes of these derangements. Using hypothesis-generating, high-dimensional single-cell analyses, we created comprehensive phenotypic maps of circulating CD4+ T cells. Differences between subject groups were confirmed in a large and genetically diverse cohort of CVID subjects (n = 69) by using flow cytometry, transcriptional profiling, multiplex cytokine/chemokine detection, and a suite of in vitro functional assays measuring naive T-cell differentiation, B-cell/T-cell cocultures, and regulatory T-cell suppression. Although CD4+ TH cell profiles from healthy donors and CVID subjects without AICs were virtually indistinguishable, T cells from CVID+AIC subjects exhibited follicular features as early as thymic egress. Follicular skewing correlated with IgA deficiency–associated endotoxemia and endotoxin-induced expression of activin A and inducible T-cell costimulator ligand. The resulting enlarged circulating follicular helper T-cell population from CVID+AIC subjects provided efficient help to receptive healthy donor B cells but not unresponsive CVID B cells. Despite this, circulating follicular helper T cells from CVID+AIC subjects exhibited aberrant transcriptional profiles and altered chemokine/cytokine receptor expression patterns that interfered with regulatory T-cell suppression assays and were associated with autoantibody production. Endotoxemia is associated with early commitment to the follicular T-cell lineage in IgA-deficient CVID subjects, particularly those with AICs. [Display omitted]
AbstractList	Although chiefly a B-lymphocyte disorder, several research groups have identified common variable immunodeficiency (CVID) subjects with numeric and/or functional TH cell alterations. The causes, interrelationships, and consequences of CVID-associated CD4+ T-cell derangements to hypogammaglobulinemia, autoantibody production, or both remain unclear. We sought to determine how circulating CD4+ T cells are altered in CVID subjects with autoimmune cytopenias (AICs; CVID+AIC) and the causes of these derangements. Using hypothesis-generating, high-dimensional single-cell analyses, we created comprehensive phenotypic maps of circulating CD4+ T cells. Differences between subject groups were confirmed in a large and genetically diverse cohort of CVID subjects (n = 69) by using flow cytometry, transcriptional profiling, multiplex cytokine/chemokine detection, and a suite of in vitro functional assays measuring naive T-cell differentiation, B-cell/T-cell cocultures, and regulatory T-cell suppression. Although CD4+ TH cell profiles from healthy donors and CVID subjects without AICs were virtually indistinguishable, T cells from CVID+AIC subjects exhibited follicular features as early as thymic egress. Follicular skewing correlated with IgA deficiency–associated endotoxemia and endotoxin-induced expression of activin A and inducible T-cell costimulator ligand. The resulting enlarged circulating follicular helper T-cell population from CVID+AIC subjects provided efficient help to receptive healthy donor B cells but not unresponsive CVID B cells. Despite this, circulating follicular helper T cells from CVID+AIC subjects exhibited aberrant transcriptional profiles and altered chemokine/cytokine receptor expression patterns that interfered with regulatory T-cell suppression assays and were associated with autoantibody production. Endotoxemia is associated with early commitment to the follicular T-cell lineage in IgA-deficient CVID subjects, particularly those with AICs. [Display omitted] Although chiefly a B-lymphocyte disorder, several research groups have identified common variable immunodeficiency (CVID) subjects with numeric and/or functional T cell alterations. The causes, interrelationships, and consequences of CVID-associated CD4 T-cell derangements to hypogammaglobulinemia, autoantibody production, or both remain unclear. We sought to determine how circulating CD4 T cells are altered in CVID subjects with autoimmune cytopenias (AICs; CVID+AIC) and the causes of these derangements. Using hypothesis-generating, high-dimensional single-cell analyses, we created comprehensive phenotypic maps of circulating CD4 T cells. Differences between subject groups were confirmed in a large and genetically diverse cohort of CVID subjects (n = 69) by using flow cytometry, transcriptional profiling, multiplex cytokine/chemokine detection, and a suite of in vitro functional assays measuring naive T-cell differentiation, B-cell/T-cell cocultures, and regulatory T-cell suppression. Although CD4 T cell profiles from healthy donors and CVID subjects without AICs were virtually indistinguishable, T cells from CVID+AIC subjects exhibited follicular features as early as thymic egress. Follicular skewing correlated with IgA deficiency-associated endotoxemia and endotoxin-induced expression of activin A and inducible T-cell costimulator ligand. The resulting enlarged circulating follicular helper T-cell population from CVID+AIC subjects provided efficient help to receptive healthy donor B cells but not unresponsive CVID B cells. Despite this, circulating follicular helper T cells from CVID+AIC subjects exhibited aberrant transcriptional profiles and altered chemokine/cytokine receptor expression patterns that interfered with regulatory T-cell suppression assays and were associated with autoantibody production. Endotoxemia is associated with early commitment to the follicular T-cell lineage in IgA-deficient CVID subjects, particularly those with AICs. BackgroundAlthough chiefly a B-lymphocyte disorder, several research groups have identified common variable immunodeficiency (CVID) subjects with numeric and/or functional TH cell alterations. The causes, interrelationships, and consequences of CVID-associated CD4+ T-cell derangements to hypogammaglobulinemia, autoantibody production, or both remain unclear.ObjectiveWe sought to determine how circulating CD4+ T cells are altered in CVID subjects with autoimmune cytopenias (AICs; CVID+AIC) and the causes of these derangements.MethodsUsing hypothesis-generating, high-dimensional single-cell analyses, we created comprehensive phenotypic maps of circulating CD4+ T cells. Differences between subject groups were confirmed in a large and genetically diverse cohort of CVID subjects (n = 69) by using flow cytometry, transcriptional profiling, multiplex cytokine/chemokine detection, and a suite of in vitro functional assays measuring naive T-cell differentiation, B-cell/T-cell cocultures, and regulatory T-cell suppression.ResultsAlthough CD4+ TH cell profiles from healthy donors and CVID subjects without AICs were virtually indistinguishable, T cells from CVID+AIC subjects exhibited follicular features as early as thymic egress. Follicular skewing correlated with IgA deficiency–associated endotoxemia and endotoxin-induced expression of activin A and inducible T-cell costimulator ligand. The resulting enlarged circulating follicular helper T-cell population from CVID+AIC subjects provided efficient help to receptive healthy donor B cells but not unresponsive CVID B cells. Despite this, circulating follicular helper T cells from CVID+AIC subjects exhibited aberrant transcriptional profiles and altered chemokine/cytokine receptor expression patterns that interfered with regulatory T-cell suppression assays and were associated with autoantibody production.ConclusionsEndotoxemia is associated with early commitment to the follicular T-cell lineage in IgA-deficient CVID subjects, particularly those with AICs. Endotoxin and plasma from IgA-deficient common variable immunodeficiency subjects with autoimmune cytopenias promote enlarged and altered T follicular helper cell populations. Although chiefly a B-lymphocyte disorder, several research groups have identified common variable immunodeficiency (CVID) subjects with numeric and/or functional TH cell alterations. The causes, interrelationships, and consequences of CVID-associated CD4+ T-cell derangements to hypogammaglobulinemia, autoantibody production, or both remain unclear.BACKGROUNDAlthough chiefly a B-lymphocyte disorder, several research groups have identified common variable immunodeficiency (CVID) subjects with numeric and/or functional TH cell alterations. The causes, interrelationships, and consequences of CVID-associated CD4+ T-cell derangements to hypogammaglobulinemia, autoantibody production, or both remain unclear.We sought to determine how circulating CD4+ T cells are altered in CVID subjects with autoimmune cytopenias (AICs; CVID+AIC) and the causes of these derangements.OBJECTIVEWe sought to determine how circulating CD4+ T cells are altered in CVID subjects with autoimmune cytopenias (AICs; CVID+AIC) and the causes of these derangements.Using hypothesis-generating, high-dimensional single-cell analyses, we created comprehensive phenotypic maps of circulating CD4+ T cells. Differences between subject groups were confirmed in a large and genetically diverse cohort of CVID subjects (n = 69) by using flow cytometry, transcriptional profiling, multiplex cytokine/chemokine detection, and a suite of in vitro functional assays measuring naive T-cell differentiation, B-cell/T-cell cocultures, and regulatory T-cell suppression.METHODSUsing hypothesis-generating, high-dimensional single-cell analyses, we created comprehensive phenotypic maps of circulating CD4+ T cells. Differences between subject groups were confirmed in a large and genetically diverse cohort of CVID subjects (n = 69) by using flow cytometry, transcriptional profiling, multiplex cytokine/chemokine detection, and a suite of in vitro functional assays measuring naive T-cell differentiation, B-cell/T-cell cocultures, and regulatory T-cell suppression.Although CD4+ TH cell profiles from healthy donors and CVID subjects without AICs were virtually indistinguishable, T cells from CVID+AIC subjects exhibited follicular features as early as thymic egress. Follicular skewing correlated with IgA deficiency-associated endotoxemia and endotoxin-induced expression of activin A and inducible T-cell costimulator ligand. The resulting enlarged circulating follicular helper T-cell population from CVID+AIC subjects provided efficient help to receptive healthy donor B cells but not unresponsive CVID B cells. Despite this, circulating follicular helper T cells from CVID+AIC subjects exhibited aberrant transcriptional profiles and altered chemokine/cytokine receptor expression patterns that interfered with regulatory T-cell suppression assays and were associated with autoantibody production.RESULTSAlthough CD4+ TH cell profiles from healthy donors and CVID subjects without AICs were virtually indistinguishable, T cells from CVID+AIC subjects exhibited follicular features as early as thymic egress. Follicular skewing correlated with IgA deficiency-associated endotoxemia and endotoxin-induced expression of activin A and inducible T-cell costimulator ligand. The resulting enlarged circulating follicular helper T-cell population from CVID+AIC subjects provided efficient help to receptive healthy donor B cells but not unresponsive CVID B cells. Despite this, circulating follicular helper T cells from CVID+AIC subjects exhibited aberrant transcriptional profiles and altered chemokine/cytokine receptor expression patterns that interfered with regulatory T-cell suppression assays and were associated with autoantibody production.Endotoxemia is associated with early commitment to the follicular T-cell lineage in IgA-deficient CVID subjects, particularly those with AICs.CONCLUSIONSEndotoxemia is associated with early commitment to the follicular T-cell lineage in IgA-deficient CVID subjects, particularly those with AICs.
Author	Henrickson, Sarah E. Kim, Taylor Olmsted Khanna, Caroline Le Coz, Carole Lambert, Michele P. Feldman, Scott Ruffner, Melanie Jyonouchi, Soma Wherry, E. John Trofa, Melissa Fadugba, Olajumoke O. Bengsch, Bertram Romberg, Neil Despotovic, Jenny M. Sullivan, Kathleen E. Nolan, Brian E. Heimall, Jennifer Ohtani, Takuya Takach, Patricia
AuthorAffiliation	g Institute for Immunology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia b Division of Hematology, Children’s Hospital of Philadelphia f Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia i Department of Pediatrics, Hematology/Oncology Section, Baylor College of Medicine, Houston, Texas c Division of Rheumatology, Children’s Hospital of Philadelphia a Division of Immunology and Allergy, Children’s Hospital of Philadelphia d Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia e Department of Medicine, Divisions of Allergy and Immunology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia h Department of Medicine II, University Medical Center Freiburg, Freiburg, Germany
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BackLink	https://www.ncbi.nlm.nih.gov/pubmed/31445098$$D View this record in MEDLINE/PubMed
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Copyright	2019 American Academy of Allergy, Asthma & Immunology Copyright © 2019 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved. 2019. American Academy of Allergy, Asthma & Immunology
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IsDoiOpenAccess	false
IsOpenAccess	true
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IsScholarly	true
Issue	6
Keywords	RTE Common variable immunodeficiency CVID−AIC ITP t-SNE FACS follicular helper T cell CVID time-of-flight cytometry TFH AIC TLR ICOS CSR Treg recent thymic emigrant autoimmune cytopenias CFSE ICOSL regulatory T cell FOXP3 ES CVID+AIC activin A HD cTFH CyTOF PD-1 endotoxin AIHA
Language	English
License	Copyright © 2019 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.
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MergedId	FETCHMERGED-LOGICAL-c534t-11c0f509b83af64bbef528f450ab1b4466fe7210eaa1de467127d6f2025e2f623
Notes	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 14 ObjectType-Article-2 ObjectType-Feature-1 content type line 23 Author contributions: CLC, BB, CK, TO, MT, BEN and SEH performed experiments and analyzed data. MPL, SF, OOF, MR, SJ, JH, PT, KES, JMD, TOK and NR provided human samples. EJW and NR were responsible for study design. CLC and NR wrote the manuscript. All authors reviewed the manuscript and provided scientific input.
ORCID	0000-0002-1881-5318
OpenAccessLink	https://www.ncbi.nlm.nih.gov/pmc/articles/6900457
PMID	31445098
PQID	2321383472
PQPubID	105664
PageCount	14
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PublicationCentury	2000
PublicationDate	2019-12-01
PublicationDateYYYYMMDD	2019-12-01
PublicationDate_xml	– month: 12 year: 2019 text: 2019-12-01 day: 01
PublicationDecade	2010
PublicationPlace	United States
PublicationPlace_xml	– name: United States – name: St. Louis
PublicationTitle	Journal of allergy and clinical immunology
PublicationTitleAlternate	J Allergy Clin Immunol
PublicationYear	2019
Publisher	Elsevier Inc Elsevier Limited
Publisher_xml	– name: Elsevier Inc – name: Elsevier Limited
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Snippet	Although chiefly a B-lymphocyte disorder, several research groups have identified common variable immunodeficiency (CVID) subjects with numeric and/or... BackgroundAlthough chiefly a B-lymphocyte disorder, several research groups have identified common variable immunodeficiency (CVID) subjects with numeric... Endotoxin and plasma from IgA-deficient common variable immunodeficiency subjects with autoimmune cytopenias promote enlarged and altered T follicular helper...
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SubjectTerms	Activin activin A Age Anemia Autoantibodies autoimmune cytopenias CD4 antigen Cell differentiation Cell lineage Chemokines Common variable immunodeficiency Costimulator Cytokines Cytopenia Endotoxemia endotoxin Enrollments Flow cytometry follicular helper T cell Hypogammaglobulinemia Immunoglobulin A Lymphocytes B Lymphocytes T Pathogenesis recent thymic emigrant regulatory T cell Signal transduction Software Thymus time-of-flight cytometry Transcription
Title	Common variable immunodeficiency–associated endotoxemia promotes early commitment to the T follicular lineage
URI	https://www.clinicalkey.com/#!/content/1-s2.0-S0091674919310929 https://dx.doi.org/10.1016/j.jaci.2019.08.007 https://www.ncbi.nlm.nih.gov/pubmed/31445098 https://www.proquest.com/docview/2321383472 https://www.proquest.com/docview/2286950331 https://pubmed.ncbi.nlm.nih.gov/PMC6900457
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