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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Veröffentlicht in:Journal of allergy and clinical immunology Jg. 144; H. 6; S. 1660 - 1673
Hauptverfasser: 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
Format: Journal Article
Sprache:Englisch
Veröffentlicht: United States Elsevier Inc 01.12.2019
Elsevier Limited
Schlagworte:
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
Online-Zugang:Volltext
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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.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.
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.
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.
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. 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]
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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  organization: Division of Immunology and Allergy, Children's Hospital of Philadelphia, Philadelphia, Pa
BackLink https://www.ncbi.nlm.nih.gov/pubmed/31445098$$D View this record in MEDLINE/PubMed
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Cites_doi 10.1182/blood-2010-08-302984
10.1016/j.immuni.2013.08.031
10.1016/j.jaci.2018.06.012
10.1186/cc13902
10.1073/pnas.0903543106
10.1056/NEJMoa1512234
10.4049/jimmunol.1402690
10.1016/S1081-1206(10)61416-0
10.1038/icb.2009.64
10.1073/pnas.0705971104
10.1182/blood-2008-07-162503
10.1371/journal.pone.0169100
10.1002/cyto.a.22625
10.1016/j.jaci.2017.04.041
10.1016/j.immuni.2012.02.012
10.1016/j.jaci.2009.05.019
10.1073/pnas.0506580102
10.1016/j.jaci.2014.02.034
10.1084/jem.20140039
10.1182/blood-2011-09-373969
10.1084/jem.20060772
10.3181/00379727-90-22032
10.1172/JCI39748
10.3389/fimmu.2018.01715
10.1007/s10875-017-0465-8
10.1016/j.immuni.2010.12.012
10.1038/ni.3226
10.1038/labinvest.3700453
10.1182/blood.V99.5.1544
10.4049/jimmunol.174.8.5074
10.1084/jem.137.4.943
10.1016/j.immuni.2011.03.023
10.4049/jimmunol.168.9.4531
10.1016/j.clim.2008.12.006
10.1126/scitranslmed.aab1216
10.1084/jem.20011756
10.1073/pnas.0400171101
10.1016/j.immuni.2018.04.026
10.1006/clim.1999.4799
10.4049/jimmunol.177.7.4927
10.1038/35051100
10.1016/j.jaci.2014.10.019
10.3324/haematol.2010.036053
10.1084/jem.192.11.1553
10.1182/blood-2010-11-321695
10.1016/j.immuni.2015.05.012
10.1182/blood-2017-03-754119
10.1016/j.jaci.2006.01.038
10.1182/blood-2009-04-215368
10.1164/rccm.201108-1560OC
10.1038/ng1180
10.1016/j.ajhg.2013.09.009
10.1016/j.ajhg.2015.07.008
10.1016/j.jaci.2015.05.036
10.1016/j.jaci.2016.08.030
10.1371/journal.pone.0074893
10.1084/jem.20190493
10.1016/j.immuni.2009.04.016
10.1172/JCI69854
10.1084/jem.192.11.1545
10.1182/asheducation.V2012.1.301.3798316
10.1126/sciimmunol.aan1487
10.1371/journal.pone.0017739
10.1126/science.1255904
10.1046/j.1365-2249.2002.01893.x
10.4049/jimmunol.164.12.6322
10.1038/ni.3494
10.1182/blood-2017-05-782177
10.1016/0092-8674(88)90379-0
10.1016/j.jaci.2011.09.027
10.1016/j.immuni.2014.04.011
10.1016/j.jaci.2015.02.022
10.3389/fimmu.2018.00636
ContentType Journal Article
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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ISSN 0091-6749
1097-6825
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Sun Apr 06 06:54:02 EDT 2025
Tue Oct 14 19:32:49 EDT 2025
IsDoiOpenAccess false
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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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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
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References Fonseca, Agua-Doce, Maceiras, Pierson, Ribeiro, Romao (bib57) 2017; 2
Unger, Seidl, van Schouwenburg, Rakhmanov, Bulashevska, Frede (bib27) 2018; 141
Alkhairy, Perez-Becker, Driessen, Abolhassani, van Montfrans, Borte (bib21) 2015; 136
Liu, Putnam, Xu-Yu, Szot, Lee, Zhu (bib46) 2006; 203
Charbonnier, Janssen, Chou, Ohsumi, Keles, Hsu (bib72) 2015; 135
Romberg, Le Coz, Glauzy, Schickel, Trofa, Nolan (bib8) 2019; 143
Bousfiha, Jeddane, Picard, Ailal, Bobby Gaspar, Al-Herz (bib34) 2018; 38
Subramanian, Tamayo, Mootha, Mukherjee, Ebert, Gillette (bib39) 2005; 102
Neunert, Lim, Crowther, Cohen, Solberg, Crowther (bib30) 2011; 117
Aladjidi, Leverger, Leblanc, Picat, Michel, Bertrand (bib32) 2011; 96
de Valles-Ibanez, Esteve-Sole, Piquer, Gonzalez-Navarro, Hernandez-Rodriguez, Laayouni (bib18) 2018; 9
Simchoni, Cunningham-Rundles (bib4) 2015; 194
Salzer, Kansu, Sic, Majek, Ikinciogullari, Dogu (bib22) 2014; 133
Castigli, Geha (bib6) 2006; 117
Schmitt, Morita, Bourdery, Bentebibel, Zurawski, Banchereau (bib48) 2009; 31
Jacquemin, Schmitt, Contin-Bordes, Liu, Narayanan, Seneschal (bib52) 2015; 42
Le Coz, Nolan, Trofa, Kamsheh, Khokha, Lakhani (bib73) 2018; 9
Locci, Wu, Arumemi, Mikulski, Dahlberg, Miller (bib50) 2016; 17
Hedl, Lahiri, Ning, Cho, Abraham (bib55) 2014; 40
Herzig, Luan, Bohannon, Toliver-Kinsky, Guo, Sherwood (bib66) 2014; 18
Guazzi, Aiuti, Mezzaroma, Mazzetta, Andolfi, Mortellaro (bib25) 2002; 129
Yu, Chiang, Song, Hoyte, Huang, Vanishsarn (bib26) 2009; 131
Kimmig, Przybylski, Schmidt, Laurisch, Mowes, Radbruch (bib47) 2002; 195
Widney, Xia, Lusis, Smith (bib63) 2000; 164
Lang, Li, Wang, Sun, Xue, Xiao (bib67) 2017; 12
Kuehn, Ouyang, Lo, Deenick, Niemela, Avery (bib71) 2014; 345
Ballesteros-Tato, Leon, Graf, Moquin, Adams, Lund (bib45) 2012; 36
Kuehn, Niemela, Sreedhara, Stoddard, Grossman, Wysocki (bib16) 2017; 130
van der Maaten, Hinton (bib36) 2008; 9
Locci, Havenar-Daughton, Landais, Wu, Kroenke, Arlehamn (bib56) 2013; 39
Ma, Wong, Rao, Avery, Torpy, Hambridge (bib69) 2015; 136
Breitfeld, Ohl, Kremmer, Ellwart, Sallusto, Lipp (bib42) 2000; 192
Lutzker, Rothman, Pollock, Coffman, Alt (bib60) 1988; 53
Thiel, Kimmig, Salzer, Grudzien, Lebrecht, Hagena (bib20) 2012; 129
Isgro, Marziali, Mezzaroma, Luzi, Mazzone, Guazzi (bib24) 2005; 174
Herzig, Driver, Fang, Toliver-Kinsky, Shute, Sherwood (bib65) 2012; 185
Dong, Juedes, Temann, Shresta, Allison, Ruddle (bib51) 2001; 409
Van Gassen, Callebaut, Van Helden, Lambrecht, Demeester, Dhaene (bib37) 2015; 87
Morita, Schmitt, Bentebibel, Ranganathan, Bourdery, Zurawski (bib44) 2011; 34
Eto, Lao, DiToro, Barnett, Escobar, Kageyama (bib49) 2011; 6
Andersson, Melchers, Galanos, Luderitz (bib59) 1973; 137
Roskin, Simchoni, Liu, Lee, Seo, Hoh (bib9) 2015; 7
Schaerli, Willimann, Lang, Lipp, Loetscher, Moser (bib43) 2000; 192
Park, Munagala, Xu, Blankenship, Maffucci, Chaussabel (bib70) 2013; 8
Warnatz, Salzer, Rizzi, Fischer, Gutenberger, Bohm (bib14) 2009; 106
Ochtrop, Goldacker, May, Rizzi, Draeger, Hauschke (bib11) 2011; 118
Choi, Kageyama, Eto, Escobar, Johnston, Monticelli (bib40) 2011; 34
Yu, Knight, Radigan, Marron, Zhang, Sanchez-Ramon (bib5) 2009; 124
Chen, Coonrod, Kumanovics, Franks, Durtschi, Margraf (bib15) 2013; 93
De Vera, Al-Harthi, Gewurz (bib23) 2004; 93
Fliegauf, Bryant, Frede, Slade, Woon, Lehnert (bib19) 2015; 97
Perreau, Vigano, Bellanger, Pellaton, Buss, Comte (bib12) 2014; 211
Jones, Mansell, Patella, Scott, Hedger, de Kretser (bib53) 2007; 104
Almejun, Campos, Patino, Galicchio, Zelazko, Oleastro (bib10) 2017; 139
Rodeghiero, Stasi, Gernsheimer, Michel, Provan, Arnold (bib29) 2009; 113
Hornung, Rothenfusser, Britsch, Krug, Jahrsdorfer, Giese (bib61) 2002; 168
Romberg, Cunningham-Rundles, Meffre (bib2) 2011; 118
Komai-Koma, Jones, Ogg, Xu, Liew (bib62) 2004; 101
Loos, Dekeyzer, Struyf, Schutyser, Gijsbers, Gouwy (bib64) 2006; 86
Bossaller, Burger, Draeger, Grimbacher, Knoth, Plebani (bib68) 2006; 177
Michel, Chanet, Dechartres, Morin, Piette, Cirasino (bib33) 2009; 114
Ma, Suryani, Avery, Chan, Nanan, Santner-Nanan (bib54) 2009; 87
Romberg, Chamberlain, Saadoun, Gentile, Kinnunen, Ng (bib3) 2013; 123
van Zelm, Smet, Adams, Mascart, Schandene, Janssen (bib17) 2010; 120
Canete, Sweet, Gonzalez-Figueroa, Papa, Ohkura, Bolton (bib74) 2019; 216
Mootha, Lindgren, Eriksson, Subramanian, Sihag, Lehar (bib38) 2003; 34
Warnatz, Denz, Drager, Braun, Groth, Wolff-Vorbeck (bib7) 2002; 99
Cunningham-Rundles (bib1) 2012; 2012
Bengsch, Ohtani, Khan, Setty, Manne, O'Brien (bib35) 2018; 48
Choi, Gullicksrud, Xing, Zeng, Shan, Li (bib41) 2015; 16
Conley, Notarangelo, Etzioni (bib28) 1999; 93
Lambert, Gernsheimer (bib31) 2017; 129
Kuehn, Boisson, Cunningham-Rundles, Reichenbach, Stray-Pedersen, Gelfand (bib13) 2016; 374
Condie, Zak, Good (bib58) 1955; 90
Kuehn (10.1016/j.jaci.2019.08.007_bib16) 2017; 130
Thiel (10.1016/j.jaci.2019.08.007_bib20) 2012; 129
Bengsch (10.1016/j.jaci.2019.08.007_bib35) 2018; 48
Liu (10.1016/j.jaci.2019.08.007_bib46) 2006; 203
Ma (10.1016/j.jaci.2019.08.007_bib54) 2009; 87
Unger (10.1016/j.jaci.2019.08.007_bib27) 2018; 141
Kuehn (10.1016/j.jaci.2019.08.007_bib71) 2014; 345
De Vera (10.1016/j.jaci.2019.08.007_bib23) 2004; 93
Roskin (10.1016/j.jaci.2019.08.007_bib9) 2015; 7
van der Maaten (10.1016/j.jaci.2019.08.007_bib36) 2008; 9
Ballesteros-Tato (10.1016/j.jaci.2019.08.007_bib45) 2012; 36
Perreau (10.1016/j.jaci.2019.08.007_bib12) 2014; 211
Breitfeld (10.1016/j.jaci.2019.08.007_bib42) 2000; 192
Park (10.1016/j.jaci.2019.08.007_bib70) 2013; 8
Schaerli (10.1016/j.jaci.2019.08.007_bib43) 2000; 192
Choi (10.1016/j.jaci.2019.08.007_bib40) 2011; 34
Charbonnier (10.1016/j.jaci.2019.08.007_bib72) 2015; 135
Almejun (10.1016/j.jaci.2019.08.007_bib10) 2017; 139
Yu (10.1016/j.jaci.2019.08.007_bib26) 2009; 131
Lutzker (10.1016/j.jaci.2019.08.007_bib60) 1988; 53
Van Gassen (10.1016/j.jaci.2019.08.007_bib37) 2015; 87
Cunningham-Rundles (10.1016/j.jaci.2019.08.007_bib1) 2012; 2012
Bousfiha (10.1016/j.jaci.2019.08.007_bib34) 2018; 38
Subramanian (10.1016/j.jaci.2019.08.007_bib39) 2005; 102
Guazzi (10.1016/j.jaci.2019.08.007_bib25) 2002; 129
Le Coz (10.1016/j.jaci.2019.08.007_bib73) 2018; 9
Fliegauf (10.1016/j.jaci.2019.08.007_bib19) 2015; 97
Neunert (10.1016/j.jaci.2019.08.007_bib30) 2011; 117
Yu (10.1016/j.jaci.2019.08.007_bib5) 2009; 124
Jones (10.1016/j.jaci.2019.08.007_bib53) 2007; 104
Warnatz (10.1016/j.jaci.2019.08.007_bib7) 2002; 99
Kimmig (10.1016/j.jaci.2019.08.007_bib47) 2002; 195
Aladjidi (10.1016/j.jaci.2019.08.007_bib32) 2011; 96
Schmitt (10.1016/j.jaci.2019.08.007_bib48) 2009; 31
Kuehn (10.1016/j.jaci.2019.08.007_bib13) 2016; 374
Canete (10.1016/j.jaci.2019.08.007_bib74) 2019; 216
Romberg (10.1016/j.jaci.2019.08.007_bib3) 2013; 123
Andersson (10.1016/j.jaci.2019.08.007_bib59) 1973; 137
Romberg (10.1016/j.jaci.2019.08.007_bib8) 2019; 143
Komai-Koma (10.1016/j.jaci.2019.08.007_bib62) 2004; 101
Bossaller (10.1016/j.jaci.2019.08.007_bib68) 2006; 177
Lambert (10.1016/j.jaci.2019.08.007_bib31) 2017; 129
Widney (10.1016/j.jaci.2019.08.007_bib63) 2000; 164
Conley (10.1016/j.jaci.2019.08.007_bib28) 1999; 93
van Zelm (10.1016/j.jaci.2019.08.007_bib17) 2010; 120
Herzig (10.1016/j.jaci.2019.08.007_bib66) 2014; 18
Ma (10.1016/j.jaci.2019.08.007_bib69) 2015; 136
Jacquemin (10.1016/j.jaci.2019.08.007_bib52) 2015; 42
Fonseca (10.1016/j.jaci.2019.08.007_bib57) 2017; 2
Dong (10.1016/j.jaci.2019.08.007_bib51) 2001; 409
Ochtrop (10.1016/j.jaci.2019.08.007_bib11) 2011; 118
Simchoni (10.1016/j.jaci.2019.08.007_bib4) 2015; 194
Condie (10.1016/j.jaci.2019.08.007_bib58) 1955; 90
Morita (10.1016/j.jaci.2019.08.007_bib44) 2011; 34
Salzer (10.1016/j.jaci.2019.08.007_bib22) 2014; 133
Michel (10.1016/j.jaci.2019.08.007_bib33) 2009; 114
Eto (10.1016/j.jaci.2019.08.007_bib49) 2011; 6
Isgro (10.1016/j.jaci.2019.08.007_bib24) 2005; 174
Hornung (10.1016/j.jaci.2019.08.007_bib61) 2002; 168
Rodeghiero (10.1016/j.jaci.2019.08.007_bib29) 2009; 113
Herzig (10.1016/j.jaci.2019.08.007_bib65) 2012; 185
Lang (10.1016/j.jaci.2019.08.007_bib67) 2017; 12
Alkhairy (10.1016/j.jaci.2019.08.007_bib21) 2015; 136
Castigli (10.1016/j.jaci.2019.08.007_bib6) 2006; 117
Hedl (10.1016/j.jaci.2019.08.007_bib55) 2014; 40
Locci (10.1016/j.jaci.2019.08.007_bib56) 2013; 39
Romberg (10.1016/j.jaci.2019.08.007_bib2) 2011; 118
Warnatz (10.1016/j.jaci.2019.08.007_bib14) 2009; 106
Mootha (10.1016/j.jaci.2019.08.007_bib38) 2003; 34
Locci (10.1016/j.jaci.2019.08.007_bib50) 2016; 17
Loos (10.1016/j.jaci.2019.08.007_bib64) 2006; 86
de Valles-Ibanez (10.1016/j.jaci.2019.08.007_bib18) 2018; 9
Choi (10.1016/j.jaci.2019.08.007_bib41) 2015; 16
Chen (10.1016/j.jaci.2019.08.007_bib15) 2013; 93
References_xml – volume: 133
  start-page: 1651
  year: 2014
  end-page: 1659.e12
  ident: bib22
  article-title: Early-onset inflammatory bowel disease and common variable immunodeficiency-like disease caused by IL-21 deficiency
  publication-title: J Allergy Clin Immunol
– volume: 106
  start-page: 13945
  year: 2009
  end-page: 13950
  ident: bib14
  article-title: B-cell activating factor receptor deficiency is associated with an adult-onset antibody deficiency syndrome in humans
  publication-title: Proc Natl Acad Sci U S A
– volume: 409
  start-page: 97
  year: 2001
  end-page: 101
  ident: bib51
  article-title: ICOS co-stimulatory receptor is essential for T-cell activation and function
  publication-title: Nature
– volume: 164
  start-page: 6322
  year: 2000
  end-page: 6331
  ident: bib63
  article-title: The murine chemokine CXCL11 (IFN-inducible T cell alpha chemoattractant) is an IFN-gamma- and lipopolysaccharide-inducible glucocorticoid-attenuated response gene expressed in lung and other tissues during endotoxemia
  publication-title: J Immunol
– volume: 192
  start-page: 1545
  year: 2000
  end-page: 1552
  ident: bib42
  article-title: Follicular B helper T cells express CXC chemokine receptor 5, localize to B cell follicles, and support immunoglobulin production
  publication-title: J Exp Med
– volume: 87
  start-page: 590
  year: 2009
  end-page: 600
  ident: bib54
  article-title: Early commitment of naive human CD4(+) T cells to the T follicular helper (T(FH)) cell lineage is induced by IL-12
  publication-title: Immunol Cell Biol
– volume: 136
  start-page: 703
  year: 2015
  end-page: 712.e10
  ident: bib21
  article-title: Novel mutations in TNFRSF7/CD27: clinical, immunologic, and genetic characterization of human CD27 deficiency
  publication-title: J Allergy Clin Immunol
– volume: 141
  start-page: 730
  year: 2018
  end-page: 740
  ident: bib27
  article-title: The TH1 phenotype of follicular helper T cells indicates an IFN-gamma-associated immune dysregulation in patients with CD21low common variable immunodeficiency
  publication-title: J Allergy Clin Immunol
– volume: 345
  start-page: 1623
  year: 2014
  end-page: 1627
  ident: bib71
  article-title: Immune dysregulation in human subjects with heterozygous germline mutations in CTLA4
  publication-title: Science
– volume: 34
  start-page: 267
  year: 2003
  end-page: 273
  ident: bib38
  article-title: PGC-1alpha-responsive genes involved in oxidative phosphorylation are coordinately downregulated in human diabetes
  publication-title: Nat Genet
– volume: 38
  start-page: 129
  year: 2018
  end-page: 143
  ident: bib34
  article-title: The 2017 IUIS phenotypic classification for primary immunodeficiencies
  publication-title: J Clin Immunol
– volume: 40
  start-page: 734
  year: 2014
  end-page: 746
  ident: bib55
  article-title: Pattern recognition receptor signaling in human dendritic cells is enhanced by ICOS ligand and modulated by the Crohn's disease ICOSLG risk allele
  publication-title: Immunity
– volume: 2012
  start-page: 301
  year: 2012
  end-page: 305
  ident: bib1
  article-title: The many faces of common variable immunodeficiency
  publication-title: Hematol Am Soc Hematol Educ Program
– volume: 8
  start-page: e74893
  year: 2013
  ident: bib70
  article-title: Interferon signature in the blood in inflammatory common variable immune deficiency
  publication-title: PLoS One
– volume: 195
  start-page: 789
  year: 2002
  end-page: 794
  ident: bib47
  article-title: Two subsets of naive T helper cells with distinct T cell receptor excision circle content in human adult peripheral blood
  publication-title: J Exp Med
– volume: 93
  start-page: 478
  year: 2004
  end-page: 484
  ident: bib23
  article-title: Assessing thymopoiesis in patients with common variable immunodeficiency as measured by T-cell receptor excision circles
  publication-title: Ann Allergy Asthma Immunol
– volume: 117
  start-page: 4190
  year: 2011
  end-page: 4207
  ident: bib30
  article-title: The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia
  publication-title: Blood
– volume: 39
  start-page: 758
  year: 2013
  end-page: 769
  ident: bib56
  article-title: Human circulating PD-1+CXCR3-CXCR5+ memory Tfh cells are highly functional and correlate with broadly neutralizing HIV antibody responses
  publication-title: Immunity
– volume: 101
  start-page: 3029
  year: 2004
  end-page: 3034
  ident: bib62
  article-title: TLR2 is expressed on activated T cells as a costimulatory receptor
  publication-title: Proc Natl Acad Sci U S A
– volume: 113
  start-page: 2386
  year: 2009
  end-page: 2393
  ident: bib29
  article-title: Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group
  publication-title: Blood
– volume: 87
  start-page: 636
  year: 2015
  end-page: 645
  ident: bib37
  article-title: FlowSOM: Using self-organizing maps for visualization and interpretation of cytometry data
  publication-title: Cytometry A
– volume: 9
  start-page: 1715
  year: 2018
  ident: bib73
  article-title: Cytotoxic T-lymphocyte-associated protein 4 haploinsufficiency-associated inflammation can occur independently of T-cell hyperproliferation
  publication-title: Front Immunol
– volume: 374
  start-page: 1032
  year: 2016
  end-page: 1043
  ident: bib13
  article-title: Loss of B Cells in Patients with Heterozygous Mutations in IKAROS
  publication-title: N Engl J Med
– volume: 42
  start-page: 1159
  year: 2015
  end-page: 1170
  ident: bib52
  article-title: OX40 ligand contributes to human lupus pathogenesis by promoting T follicular helper response
  publication-title: Immunity
– volume: 139
  start-page: 913
  year: 2017
  end-page: 922
  ident: bib10
  article-title: Noninfectious complications in patients with pediatric-onset common variable immunodeficiency correlated with defects in somatic hypermutation but not in class-switch recombination
  publication-title: J Allergy Clin Immunol
– volume: 17
  start-page: 976
  year: 2016
  end-page: 984
  ident: bib50
  article-title: Activin A programs the differentiation of human TFH cells
  publication-title: Nat Immunol
– volume: 104
  start-page: 16239
  year: 2007
  end-page: 16244
  ident: bib53
  article-title: Activin A is a critical component of the inflammatory response, and its binding protein, follistatin, reduces mortality in endotoxemia
  publication-title: Proc Natl Acad Sci U S A
– volume: 177
  start-page: 4927
  year: 2006
  end-page: 4932
  ident: bib68
  article-title: ICOS deficiency is associated with a severe reduction of CXCR5+CD4 germinal center Th cells
  publication-title: J Immunol
– volume: 118
  start-page: 5977
  year: 2011
  end-page: 5978
  ident: bib2
  article-title: Response: common variable immunodeficiency patients with increased CD21−/lo B cells suffer from altered receptor editing and defective central B-cell tolerance
  publication-title: Blood
– volume: 9
  start-page: 2579
  year: 2008
  end-page: 2605
  ident: bib36
  article-title: Visualizing high-dimensional data using t-SNE
  publication-title: J Machine Learning Res
– volume: 123
  start-page: 4283
  year: 2013
  end-page: 4293
  ident: bib3
  article-title: CVID-associated TACI mutations affect autoreactive B cell selection and activation
  publication-title: J Clin Invest
– volume: 34
  start-page: 932
  year: 2011
  end-page: 946
  ident: bib40
  article-title: ICOS receptor instructs T follicular helper cell versus effector cell differentiation via induction of the transcriptional repressor Bcl6
  publication-title: Immunity
– volume: 34
  start-page: 108
  year: 2011
  end-page: 121
  ident: bib44
  article-title: Human blood CXCR5(+)CD4(+) T cells are counterparts of T follicular cells and contain specific subsets that differentially support antibody secretion
  publication-title: Immunity
– volume: 36
  start-page: 847
  year: 2012
  end-page: 856
  ident: bib45
  article-title: Interleukin-2 inhibits germinal center formation by limiting T follicular helper cell differentiation
  publication-title: Immunity
– volume: 124
  start-page: 349
  year: 2009
  end-page: 356.e1-3
  ident: bib5
  article-title: Toll-like receptor 7 and 9 defects in common variable immunodeficiency
  publication-title: J Allergy Clin Immunol
– volume: 9
  start-page: 636
  year: 2018
  ident: bib18
  article-title: Evaluating the genetics of common variable immunodeficiency: monogenetic model and beyond
  publication-title: Front Immunol
– volume: 174
  start-page: 5074
  year: 2005
  end-page: 5081
  ident: bib24
  article-title: Bone marrow clonogenic capability, cytokine production, and thymic output in patients with common variable immunodeficiency
  publication-title: J Immunol
– volume: 211
  start-page: 2033
  year: 2014
  end-page: 2045
  ident: bib12
  article-title: Exhaustion of bacteria-specific CD4 T cells and microbial translocation in common variable immunodeficiency disorders
  publication-title: J Exp Med
– volume: 6
  start-page: e17739
  year: 2011
  ident: bib49
  article-title: IL-21 and IL-6 are critical for different aspects of B cell immunity and redundantly induce optimal follicular helper CD4 T cell (Tfh) differentiation
  publication-title: PLoS One
– volume: 131
  start-page: 240
  year: 2009
  end-page: 253
  ident: bib26
  article-title: Regulatory T cell dysfunction in subjects with common variable immunodeficiency complicated by autoimmune disease
  publication-title: Clin Immunol
– volume: 118
  start-page: 309
  year: 2011
  end-page: 318
  ident: bib11
  article-title: T and B lymphocyte abnormalities in bone marrow biopsies of common variable immunodeficiency
  publication-title: Blood
– volume: 135
  start-page: 217
  year: 2015
  end-page: 227
  ident: bib72
  article-title: Regulatory T-cell deficiency and immune dysregulation, polyendocrinopathy, enteropathy, X-linked-like disorder caused by loss-of-function mutations in LRBA
  publication-title: J Allergy Clin Immunol
– volume: 93
  start-page: 812
  year: 2013
  end-page: 824
  ident: bib15
  article-title: Germline mutations in NFKB2 implicate the noncanonical NF-kappaB pathway in the pathogenesis of common variable immunodeficiency
  publication-title: Am J Hum Genet
– volume: 114
  start-page: 3167
  year: 2009
  end-page: 3172
  ident: bib33
  article-title: The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases
  publication-title: Blood
– volume: 16
  start-page: 980
  year: 2015
  end-page: 990
  ident: bib41
  article-title: LEF-1 and TCF-1 orchestrate T(FH) differentiation by regulating differentiation circuits upstream of the transcriptional repressor Bcl6
  publication-title: Nat Immunol
– volume: 136
  start-page: 993
  year: 2015
  end-page: 1006.e1
  ident: bib69
  article-title: Monogenic mutations differentially affect the quantity and quality of T follicular helper cells in patients with human primary immunodeficiencies
  publication-title: J Allergy Clin Immunol
– volume: 194
  start-page: 3035
  year: 2015
  end-page: 3044
  ident: bib4
  article-title: TLR7- and TLR9-responsive human B cells share phenotypic and genetic characteristics
  publication-title: J Immunol
– volume: 97
  start-page: 389
  year: 2015
  end-page: 403
  ident: bib19
  article-title: Haploinsufficiency of the NF-kappaB1 subunit p50 in common variable immunodeficiency
  publication-title: Am J Hum Genet
– volume: 2
  year: 2017
  ident: bib57
  article-title: Human blood Tfr cells are indicators of ongoing humoral activity not fully licensed with suppressive function
  publication-title: Sci Immunol
– volume: 93
  start-page: 190
  year: 1999
  end-page: 197
  ident: bib28
  article-title: Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies)
  publication-title: Clin Immunol
– volume: 31
  start-page: 158
  year: 2009
  end-page: 169
  ident: bib48
  article-title: Human dendritic cells induce the differentiation of interleukin-21-producing T follicular helper-like cells through interleukin-12
  publication-title: Immunity
– volume: 96
  start-page: 655
  year: 2011
  end-page: 663
  ident: bib32
  article-title: New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children
  publication-title: Haematologica
– volume: 168
  start-page: 4531
  year: 2002
  end-page: 4537
  ident: bib61
  article-title: Quantitative expression of toll-like receptor 1-10 mRNA in cellular subsets of human peripheral blood mononuclear cells and sensitivity to CpG oligodeoxynucleotides
  publication-title: J Immunol
– volume: 99
  start-page: 1544
  year: 2002
  end-page: 1551
  ident: bib7
  article-title: Severe deficiency of switched memory B cells (CD27(+)IgM(-)IgD(-)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease
  publication-title: Blood
– volume: 102
  start-page: 15545
  year: 2005
  end-page: 15550
  ident: bib39
  article-title: Gene set enrichment analysis: a knowledge-based approach for interpreting genome-wide expression profiles
  publication-title: Proc Natl Acad Sci U S A
– volume: 216
  start-page: 1843
  year: 2019
  end-page: 1856
  ident: bib74
  article-title: Regulatory roles of IL-10-producing human follicular T cells
  publication-title: J Exp Med
– volume: 185
  start-page: 291
  year: 2012
  end-page: 300
  ident: bib65
  article-title: Regulation of lymphocyte trafficking by CXC chemokine receptor 3 during septic shock
  publication-title: Am J Respir Crit Care Med
– volume: 120
  start-page: 1265
  year: 2010
  end-page: 1274
  ident: bib17
  article-title: CD81 gene defect in humans disrupts CD19 complex formation and leads to antibody deficiency
  publication-title: J Clin Invest
– volume: 12
  start-page: e0169100
  year: 2017
  ident: bib67
  article-title: CXCL10/IP-10 Neutralization can ameliorate lipopolysaccharide-induced acute respiratory distress syndrome in rats
  publication-title: PLoS One
– volume: 129
  start-page: 2829
  year: 2017
  end-page: 2835
  ident: bib31
  article-title: Clinical updates in adult immune thrombocytopenia
  publication-title: Blood
– volume: 7
  start-page: 302ra135
  year: 2015
  ident: bib9
  article-title: IgH sequences in common variable immune deficiency reveal altered B cell development and selection
  publication-title: Sci Transl Med
– volume: 90
  start-page: 355
  year: 1955
  end-page: 360
  ident: bib58
  article-title: Effect of meningococcal endotoxin on the immune response
  publication-title: Proc Soc Exp Biol Med
– volume: 86
  start-page: 902
  year: 2006
  end-page: 916
  ident: bib64
  article-title: TLR ligands and cytokines induce CXCR3 ligands in endothelial cells: enhanced CXCL9 in autoimmune arthritis
  publication-title: Lab Invest
– volume: 48
  start-page: 1029
  year: 2018
  end-page: 1045. e5
  ident: bib35
  article-title: Epigenomic-guided mass cytometry profiling reveals disease-specific features of exhausted CD8 T cells
  publication-title: Immunity
– volume: 129
  start-page: 346
  year: 2002
  end-page: 353
  ident: bib25
  article-title: Assessment of thymic output in common variable immunodeficiency patients by evaluation of T cell receptor excision circles
  publication-title: Clin Exp Immunol
– volume: 137
  start-page: 943
  year: 1973
  end-page: 953
  ident: bib59
  article-title: The mitogenic effect of lipopolysaccharide on bone marrow-derived mouse lymphocytes. Lipid A as the mitogenic part of the molecule
  publication-title: J Exp Med
– volume: 130
  start-page: 1553
  year: 2017
  end-page: 1564
  ident: bib16
  article-title: Novel nonsense gain-of-function NFKB2 mutations associated with a combined immunodeficiency phenotype
  publication-title: Blood
– volume: 203
  start-page: 1701
  year: 2006
  end-page: 1711
  ident: bib46
  article-title: CD127 expression inversely correlates with FoxP3 and suppressive function of human CD4+ T reg cells
  publication-title: J Exp Med
– volume: 18
  start-page: R113
  year: 2014
  ident: bib66
  article-title: The role of CXCL10 in the pathogenesis of experimental septic shock
  publication-title: Crit Care
– volume: 117
  start-page: 740
  year: 2006
  end-page: 747
  ident: bib6
  article-title: Molecular basis of common variable immunodeficiency
  publication-title: J Allergy Clin Immunol
– volume: 192
  start-page: 1553
  year: 2000
  end-page: 1562
  ident: bib43
  article-title: CXC chemokine receptor 5 expression defines follicular homing T cells with B cell helper function
  publication-title: J Exp Med
– volume: 129
  start-page: 801
  year: 2012
  end-page: 810.e6
  ident: bib20
  article-title: Genetic CD21 deficiency is associated with hypogammaglobulinemia
  publication-title: J Allergy Clin Immunol
– volume: 53
  start-page: 177
  year: 1988
  end-page: 184
  ident: bib60
  article-title: Mitogen- and IL-4-regulated expression of germ-line Ig gamma 2b transcripts: evidence for directed heavy chain class switching
  publication-title: Cell
– volume: 143
  start-page: 258
  year: 2019
  end-page: 265
  ident: bib8
  article-title: Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses
  publication-title: J Allergy Clin Immunol
– volume: 117
  start-page: 4190
  year: 2011
  ident: 10.1016/j.jaci.2019.08.007_bib30
  article-title: The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia
  publication-title: Blood
  doi: 10.1182/blood-2010-08-302984
– volume: 39
  start-page: 758
  year: 2013
  ident: 10.1016/j.jaci.2019.08.007_bib56
  article-title: Human circulating PD-1+CXCR3-CXCR5+ memory Tfh cells are highly functional and correlate with broadly neutralizing HIV antibody responses
  publication-title: Immunity
  doi: 10.1016/j.immuni.2013.08.031
– volume: 143
  start-page: 258
  year: 2019
  ident: 10.1016/j.jaci.2019.08.007_bib8
  article-title: Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses
  publication-title: J Allergy Clin Immunol
  doi: 10.1016/j.jaci.2018.06.012
– volume: 18
  start-page: R113
  year: 2014
  ident: 10.1016/j.jaci.2019.08.007_bib66
  article-title: The role of CXCL10 in the pathogenesis of experimental septic shock
  publication-title: Crit Care
  doi: 10.1186/cc13902
– volume: 106
  start-page: 13945
  year: 2009
  ident: 10.1016/j.jaci.2019.08.007_bib14
  article-title: B-cell activating factor receptor deficiency is associated with an adult-onset antibody deficiency syndrome in humans
  publication-title: Proc Natl Acad Sci U S A
  doi: 10.1073/pnas.0903543106
– volume: 374
  start-page: 1032
  year: 2016
  ident: 10.1016/j.jaci.2019.08.007_bib13
  article-title: Loss of B Cells in Patients with Heterozygous Mutations in IKAROS
  publication-title: N Engl J Med
  doi: 10.1056/NEJMoa1512234
– volume: 194
  start-page: 3035
  year: 2015
  ident: 10.1016/j.jaci.2019.08.007_bib4
  article-title: TLR7- and TLR9-responsive human B cells share phenotypic and genetic characteristics
  publication-title: J Immunol
  doi: 10.4049/jimmunol.1402690
– volume: 93
  start-page: 478
  year: 2004
  ident: 10.1016/j.jaci.2019.08.007_bib23
  article-title: Assessing thymopoiesis in patients with common variable immunodeficiency as measured by T-cell receptor excision circles
  publication-title: Ann Allergy Asthma Immunol
  doi: 10.1016/S1081-1206(10)61416-0
– volume: 87
  start-page: 590
  year: 2009
  ident: 10.1016/j.jaci.2019.08.007_bib54
  article-title: Early commitment of naive human CD4(+) T cells to the T follicular helper (T(FH)) cell lineage is induced by IL-12
  publication-title: Immunol Cell Biol
  doi: 10.1038/icb.2009.64
– volume: 104
  start-page: 16239
  year: 2007
  ident: 10.1016/j.jaci.2019.08.007_bib53
  article-title: Activin A is a critical component of the inflammatory response, and its binding protein, follistatin, reduces mortality in endotoxemia
  publication-title: Proc Natl Acad Sci U S A
  doi: 10.1073/pnas.0705971104
– volume: 113
  start-page: 2386
  year: 2009
  ident: 10.1016/j.jaci.2019.08.007_bib29
  article-title: Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group
  publication-title: Blood
  doi: 10.1182/blood-2008-07-162503
– volume: 12
  start-page: e0169100
  year: 2017
  ident: 10.1016/j.jaci.2019.08.007_bib67
  article-title: CXCL10/IP-10 Neutralization can ameliorate lipopolysaccharide-induced acute respiratory distress syndrome in rats
  publication-title: PLoS One
  doi: 10.1371/journal.pone.0169100
– volume: 87
  start-page: 636
  year: 2015
  ident: 10.1016/j.jaci.2019.08.007_bib37
  article-title: FlowSOM: Using self-organizing maps for visualization and interpretation of cytometry data
  publication-title: Cytometry A
  doi: 10.1002/cyto.a.22625
– volume: 141
  start-page: 730
  year: 2018
  ident: 10.1016/j.jaci.2019.08.007_bib27
  article-title: The TH1 phenotype of follicular helper T cells indicates an IFN-gamma-associated immune dysregulation in patients with CD21low common variable immunodeficiency
  publication-title: J Allergy Clin Immunol
  doi: 10.1016/j.jaci.2017.04.041
– volume: 36
  start-page: 847
  year: 2012
  ident: 10.1016/j.jaci.2019.08.007_bib45
  article-title: Interleukin-2 inhibits germinal center formation by limiting T follicular helper cell differentiation
  publication-title: Immunity
  doi: 10.1016/j.immuni.2012.02.012
– volume: 124
  start-page: 349
  year: 2009
  ident: 10.1016/j.jaci.2019.08.007_bib5
  article-title: Toll-like receptor 7 and 9 defects in common variable immunodeficiency
  publication-title: J Allergy Clin Immunol
  doi: 10.1016/j.jaci.2009.05.019
– volume: 102
  start-page: 15545
  year: 2005
  ident: 10.1016/j.jaci.2019.08.007_bib39
  article-title: Gene set enrichment analysis: a knowledge-based approach for interpreting genome-wide expression profiles
  publication-title: Proc Natl Acad Sci U S A
  doi: 10.1073/pnas.0506580102
– volume: 133
  start-page: 1651
  year: 2014
  ident: 10.1016/j.jaci.2019.08.007_bib22
  article-title: Early-onset inflammatory bowel disease and common variable immunodeficiency-like disease caused by IL-21 deficiency
  publication-title: J Allergy Clin Immunol
  doi: 10.1016/j.jaci.2014.02.034
– volume: 211
  start-page: 2033
  year: 2014
  ident: 10.1016/j.jaci.2019.08.007_bib12
  article-title: Exhaustion of bacteria-specific CD4 T cells and microbial translocation in common variable immunodeficiency disorders
  publication-title: J Exp Med
  doi: 10.1084/jem.20140039
– volume: 118
  start-page: 5977
  year: 2011
  ident: 10.1016/j.jaci.2019.08.007_bib2
  article-title: Response: common variable immunodeficiency patients with increased CD21−/lo B cells suffer from altered receptor editing and defective central B-cell tolerance
  publication-title: Blood
  doi: 10.1182/blood-2011-09-373969
– volume: 203
  start-page: 1701
  year: 2006
  ident: 10.1016/j.jaci.2019.08.007_bib46
  article-title: CD127 expression inversely correlates with FoxP3 and suppressive function of human CD4+ T reg cells
  publication-title: J Exp Med
  doi: 10.1084/jem.20060772
– volume: 90
  start-page: 355
  year: 1955
  ident: 10.1016/j.jaci.2019.08.007_bib58
  article-title: Effect of meningococcal endotoxin on the immune response
  publication-title: Proc Soc Exp Biol Med
  doi: 10.3181/00379727-90-22032
– volume: 120
  start-page: 1265
  year: 2010
  ident: 10.1016/j.jaci.2019.08.007_bib17
  article-title: CD81 gene defect in humans disrupts CD19 complex formation and leads to antibody deficiency
  publication-title: J Clin Invest
  doi: 10.1172/JCI39748
– volume: 9
  start-page: 1715
  year: 2018
  ident: 10.1016/j.jaci.2019.08.007_bib73
  article-title: Cytotoxic T-lymphocyte-associated protein 4 haploinsufficiency-associated inflammation can occur independently of T-cell hyperproliferation
  publication-title: Front Immunol
  doi: 10.3389/fimmu.2018.01715
– volume: 38
  start-page: 129
  year: 2018
  ident: 10.1016/j.jaci.2019.08.007_bib34
  article-title: The 2017 IUIS phenotypic classification for primary immunodeficiencies
  publication-title: J Clin Immunol
  doi: 10.1007/s10875-017-0465-8
– volume: 34
  start-page: 108
  year: 2011
  ident: 10.1016/j.jaci.2019.08.007_bib44
  article-title: Human blood CXCR5(+)CD4(+) T cells are counterparts of T follicular cells and contain specific subsets that differentially support antibody secretion
  publication-title: Immunity
  doi: 10.1016/j.immuni.2010.12.012
– volume: 16
  start-page: 980
  year: 2015
  ident: 10.1016/j.jaci.2019.08.007_bib41
  article-title: LEF-1 and TCF-1 orchestrate T(FH) differentiation by regulating differentiation circuits upstream of the transcriptional repressor Bcl6
  publication-title: Nat Immunol
  doi: 10.1038/ni.3226
– volume: 86
  start-page: 902
  year: 2006
  ident: 10.1016/j.jaci.2019.08.007_bib64
  article-title: TLR ligands and cytokines induce CXCR3 ligands in endothelial cells: enhanced CXCL9 in autoimmune arthritis
  publication-title: Lab Invest
  doi: 10.1038/labinvest.3700453
– volume: 99
  start-page: 1544
  year: 2002
  ident: 10.1016/j.jaci.2019.08.007_bib7
  article-title: Severe deficiency of switched memory B cells (CD27(+)IgM(-)IgD(-)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease
  publication-title: Blood
  doi: 10.1182/blood.V99.5.1544
– volume: 174
  start-page: 5074
  year: 2005
  ident: 10.1016/j.jaci.2019.08.007_bib24
  article-title: Bone marrow clonogenic capability, cytokine production, and thymic output in patients with common variable immunodeficiency
  publication-title: J Immunol
  doi: 10.4049/jimmunol.174.8.5074
– volume: 137
  start-page: 943
  year: 1973
  ident: 10.1016/j.jaci.2019.08.007_bib59
  article-title: The mitogenic effect of lipopolysaccharide on bone marrow-derived mouse lymphocytes. Lipid A as the mitogenic part of the molecule
  publication-title: J Exp Med
  doi: 10.1084/jem.137.4.943
– volume: 34
  start-page: 932
  year: 2011
  ident: 10.1016/j.jaci.2019.08.007_bib40
  article-title: ICOS receptor instructs T follicular helper cell versus effector cell differentiation via induction of the transcriptional repressor Bcl6
  publication-title: Immunity
  doi: 10.1016/j.immuni.2011.03.023
– volume: 168
  start-page: 4531
  year: 2002
  ident: 10.1016/j.jaci.2019.08.007_bib61
  article-title: Quantitative expression of toll-like receptor 1-10 mRNA in cellular subsets of human peripheral blood mononuclear cells and sensitivity to CpG oligodeoxynucleotides
  publication-title: J Immunol
  doi: 10.4049/jimmunol.168.9.4531
– volume: 131
  start-page: 240
  year: 2009
  ident: 10.1016/j.jaci.2019.08.007_bib26
  article-title: Regulatory T cell dysfunction in subjects with common variable immunodeficiency complicated by autoimmune disease
  publication-title: Clin Immunol
  doi: 10.1016/j.clim.2008.12.006
– volume: 7
  start-page: 302ra135
  year: 2015
  ident: 10.1016/j.jaci.2019.08.007_bib9
  article-title: IgH sequences in common variable immune deficiency reveal altered B cell development and selection
  publication-title: Sci Transl Med
  doi: 10.1126/scitranslmed.aab1216
– volume: 195
  start-page: 789
  year: 2002
  ident: 10.1016/j.jaci.2019.08.007_bib47
  article-title: Two subsets of naive T helper cells with distinct T cell receptor excision circle content in human adult peripheral blood
  publication-title: J Exp Med
  doi: 10.1084/jem.20011756
– volume: 101
  start-page: 3029
  year: 2004
  ident: 10.1016/j.jaci.2019.08.007_bib62
  article-title: TLR2 is expressed on activated T cells as a costimulatory receptor
  publication-title: Proc Natl Acad Sci U S A
  doi: 10.1073/pnas.0400171101
– volume: 48
  start-page: 1029
  year: 2018
  ident: 10.1016/j.jaci.2019.08.007_bib35
  article-title: Epigenomic-guided mass cytometry profiling reveals disease-specific features of exhausted CD8 T cells
  publication-title: Immunity
  doi: 10.1016/j.immuni.2018.04.026
– volume: 93
  start-page: 190
  year: 1999
  ident: 10.1016/j.jaci.2019.08.007_bib28
  article-title: Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies)
  publication-title: Clin Immunol
  doi: 10.1006/clim.1999.4799
– volume: 177
  start-page: 4927
  year: 2006
  ident: 10.1016/j.jaci.2019.08.007_bib68
  article-title: ICOS deficiency is associated with a severe reduction of CXCR5+CD4 germinal center Th cells
  publication-title: J Immunol
  doi: 10.4049/jimmunol.177.7.4927
– volume: 409
  start-page: 97
  year: 2001
  ident: 10.1016/j.jaci.2019.08.007_bib51
  article-title: ICOS co-stimulatory receptor is essential for T-cell activation and function
  publication-title: Nature
  doi: 10.1038/35051100
– volume: 135
  start-page: 217
  year: 2015
  ident: 10.1016/j.jaci.2019.08.007_bib72
  article-title: Regulatory T-cell deficiency and immune dysregulation, polyendocrinopathy, enteropathy, X-linked-like disorder caused by loss-of-function mutations in LRBA
  publication-title: J Allergy Clin Immunol
  doi: 10.1016/j.jaci.2014.10.019
– volume: 96
  start-page: 655
  year: 2011
  ident: 10.1016/j.jaci.2019.08.007_bib32
  article-title: New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children
  publication-title: Haematologica
  doi: 10.3324/haematol.2010.036053
– volume: 192
  start-page: 1553
  year: 2000
  ident: 10.1016/j.jaci.2019.08.007_bib43
  article-title: CXC chemokine receptor 5 expression defines follicular homing T cells with B cell helper function
  publication-title: J Exp Med
  doi: 10.1084/jem.192.11.1553
– volume: 118
  start-page: 309
  year: 2011
  ident: 10.1016/j.jaci.2019.08.007_bib11
  article-title: T and B lymphocyte abnormalities in bone marrow biopsies of common variable immunodeficiency
  publication-title: Blood
  doi: 10.1182/blood-2010-11-321695
– volume: 42
  start-page: 1159
  year: 2015
  ident: 10.1016/j.jaci.2019.08.007_bib52
  article-title: OX40 ligand contributes to human lupus pathogenesis by promoting T follicular helper response
  publication-title: Immunity
  doi: 10.1016/j.immuni.2015.05.012
– volume: 129
  start-page: 2829
  year: 2017
  ident: 10.1016/j.jaci.2019.08.007_bib31
  article-title: Clinical updates in adult immune thrombocytopenia
  publication-title: Blood
  doi: 10.1182/blood-2017-03-754119
– volume: 117
  start-page: 740
  year: 2006
  ident: 10.1016/j.jaci.2019.08.007_bib6
  article-title: Molecular basis of common variable immunodeficiency
  publication-title: J Allergy Clin Immunol
  doi: 10.1016/j.jaci.2006.01.038
– volume: 114
  start-page: 3167
  year: 2009
  ident: 10.1016/j.jaci.2019.08.007_bib33
  article-title: The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases
  publication-title: Blood
  doi: 10.1182/blood-2009-04-215368
– volume: 185
  start-page: 291
  year: 2012
  ident: 10.1016/j.jaci.2019.08.007_bib65
  article-title: Regulation of lymphocyte trafficking by CXC chemokine receptor 3 during septic shock
  publication-title: Am J Respir Crit Care Med
  doi: 10.1164/rccm.201108-1560OC
– volume: 34
  start-page: 267
  year: 2003
  ident: 10.1016/j.jaci.2019.08.007_bib38
  article-title: PGC-1alpha-responsive genes involved in oxidative phosphorylation are coordinately downregulated in human diabetes
  publication-title: Nat Genet
  doi: 10.1038/ng1180
– volume: 93
  start-page: 812
  year: 2013
  ident: 10.1016/j.jaci.2019.08.007_bib15
  article-title: Germline mutations in NFKB2 implicate the noncanonical NF-kappaB pathway in the pathogenesis of common variable immunodeficiency
  publication-title: Am J Hum Genet
  doi: 10.1016/j.ajhg.2013.09.009
– volume: 97
  start-page: 389
  year: 2015
  ident: 10.1016/j.jaci.2019.08.007_bib19
  article-title: Haploinsufficiency of the NF-kappaB1 subunit p50 in common variable immunodeficiency
  publication-title: Am J Hum Genet
  doi: 10.1016/j.ajhg.2015.07.008
– volume: 136
  start-page: 993
  year: 2015
  ident: 10.1016/j.jaci.2019.08.007_bib69
  article-title: Monogenic mutations differentially affect the quantity and quality of T follicular helper cells in patients with human primary immunodeficiencies
  publication-title: J Allergy Clin Immunol
  doi: 10.1016/j.jaci.2015.05.036
– volume: 139
  start-page: 913
  year: 2017
  ident: 10.1016/j.jaci.2019.08.007_bib10
  article-title: Noninfectious complications in patients with pediatric-onset common variable immunodeficiency correlated with defects in somatic hypermutation but not in class-switch recombination
  publication-title: J Allergy Clin Immunol
  doi: 10.1016/j.jaci.2016.08.030
– volume: 8
  start-page: e74893
  year: 2013
  ident: 10.1016/j.jaci.2019.08.007_bib70
  article-title: Interferon signature in the blood in inflammatory common variable immune deficiency
  publication-title: PLoS One
  doi: 10.1371/journal.pone.0074893
– volume: 216
  start-page: 1843
  year: 2019
  ident: 10.1016/j.jaci.2019.08.007_bib74
  article-title: Regulatory roles of IL-10-producing human follicular T cells
  publication-title: J Exp Med
  doi: 10.1084/jem.20190493
– volume: 31
  start-page: 158
  year: 2009
  ident: 10.1016/j.jaci.2019.08.007_bib48
  article-title: Human dendritic cells induce the differentiation of interleukin-21-producing T follicular helper-like cells through interleukin-12
  publication-title: Immunity
  doi: 10.1016/j.immuni.2009.04.016
– volume: 123
  start-page: 4283
  year: 2013
  ident: 10.1016/j.jaci.2019.08.007_bib3
  article-title: CVID-associated TACI mutations affect autoreactive B cell selection and activation
  publication-title: J Clin Invest
  doi: 10.1172/JCI69854
– volume: 192
  start-page: 1545
  year: 2000
  ident: 10.1016/j.jaci.2019.08.007_bib42
  article-title: Follicular B helper T cells express CXC chemokine receptor 5, localize to B cell follicles, and support immunoglobulin production
  publication-title: J Exp Med
  doi: 10.1084/jem.192.11.1545
– volume: 2012
  start-page: 301
  year: 2012
  ident: 10.1016/j.jaci.2019.08.007_bib1
  article-title: The many faces of common variable immunodeficiency
  publication-title: Hematol Am Soc Hematol Educ Program
  doi: 10.1182/asheducation.V2012.1.301.3798316
– volume: 2
  year: 2017
  ident: 10.1016/j.jaci.2019.08.007_bib57
  article-title: Human blood Tfr cells are indicators of ongoing humoral activity not fully licensed with suppressive function
  publication-title: Sci Immunol
  doi: 10.1126/sciimmunol.aan1487
– volume: 6
  start-page: e17739
  year: 2011
  ident: 10.1016/j.jaci.2019.08.007_bib49
  article-title: IL-21 and IL-6 are critical for different aspects of B cell immunity and redundantly induce optimal follicular helper CD4 T cell (Tfh) differentiation
  publication-title: PLoS One
  doi: 10.1371/journal.pone.0017739
– volume: 345
  start-page: 1623
  year: 2014
  ident: 10.1016/j.jaci.2019.08.007_bib71
  article-title: Immune dysregulation in human subjects with heterozygous germline mutations in CTLA4
  publication-title: Science
  doi: 10.1126/science.1255904
– volume: 129
  start-page: 346
  year: 2002
  ident: 10.1016/j.jaci.2019.08.007_bib25
  article-title: Assessment of thymic output in common variable immunodeficiency patients by evaluation of T cell receptor excision circles
  publication-title: Clin Exp Immunol
  doi: 10.1046/j.1365-2249.2002.01893.x
– volume: 164
  start-page: 6322
  year: 2000
  ident: 10.1016/j.jaci.2019.08.007_bib63
  article-title: The murine chemokine CXCL11 (IFN-inducible T cell alpha chemoattractant) is an IFN-gamma- and lipopolysaccharide-inducible glucocorticoid-attenuated response gene expressed in lung and other tissues during endotoxemia
  publication-title: J Immunol
  doi: 10.4049/jimmunol.164.12.6322
– volume: 17
  start-page: 976
  year: 2016
  ident: 10.1016/j.jaci.2019.08.007_bib50
  article-title: Activin A programs the differentiation of human TFH cells
  publication-title: Nat Immunol
  doi: 10.1038/ni.3494
– volume: 130
  start-page: 1553
  year: 2017
  ident: 10.1016/j.jaci.2019.08.007_bib16
  article-title: Novel nonsense gain-of-function NFKB2 mutations associated with a combined immunodeficiency phenotype
  publication-title: Blood
  doi: 10.1182/blood-2017-05-782177
– volume: 9
  start-page: 2579
  year: 2008
  ident: 10.1016/j.jaci.2019.08.007_bib36
  article-title: Visualizing high-dimensional data using t-SNE
  publication-title: J Machine Learning Res
– volume: 53
  start-page: 177
  year: 1988
  ident: 10.1016/j.jaci.2019.08.007_bib60
  article-title: Mitogen- and IL-4-regulated expression of germ-line Ig gamma 2b transcripts: evidence for directed heavy chain class switching
  publication-title: Cell
  doi: 10.1016/0092-8674(88)90379-0
– volume: 129
  start-page: 801
  year: 2012
  ident: 10.1016/j.jaci.2019.08.007_bib20
  article-title: Genetic CD21 deficiency is associated with hypogammaglobulinemia
  publication-title: J Allergy Clin Immunol
  doi: 10.1016/j.jaci.2011.09.027
– volume: 40
  start-page: 734
  year: 2014
  ident: 10.1016/j.jaci.2019.08.007_bib55
  article-title: Pattern recognition receptor signaling in human dendritic cells is enhanced by ICOS ligand and modulated by the Crohn's disease ICOSLG risk allele
  publication-title: Immunity
  doi: 10.1016/j.immuni.2014.04.011
– volume: 136
  start-page: 703
  year: 2015
  ident: 10.1016/j.jaci.2019.08.007_bib21
  article-title: Novel mutations in TNFRSF7/CD27: clinical, immunologic, and genetic characterization of human CD27 deficiency
  publication-title: J Allergy Clin Immunol
  doi: 10.1016/j.jaci.2015.02.022
– volume: 9
  start-page: 636
  year: 2018
  ident: 10.1016/j.jaci.2019.08.007_bib18
  article-title: Evaluating the genetics of common variable immunodeficiency: monogenetic model and beyond
  publication-title: Front Immunol
  doi: 10.3389/fimmu.2018.00636
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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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StartPage 1660
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
Volume 144
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