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WARM AUTOIMMUNE HEMOLYTIC ANEMIA IS ASSOCIATED WITH A REGULATORY T CELL DEFICIENCY AND A TH17 POLARIZATION
Author(s): ,
Marion CIUDAD
Affiliations:
Department of Internal Medicine and Clinical Immunology, Referral centre for autoimmune cytopenia in adults (CeReCAI),Dijon University Hospital,DIJON,France;UMR1098, RIGHT Interactions Greffon-Hôte-Tumeur/Ingénierie Cellulaire et Génique,Université Bourgogne Franche-Comté, INSERM, EFS BFC,DIJON,France
,
Sethi OUANDJI
Affiliations:
Department of Internal Medicine and Clinical Immunology, Referral centre for autoimmune cytopenia in adults (CeReCAI),Dijon University Hospital,DIJON,France
,
Claudie CLADIERE
Affiliations:
Department of Internal Medicine and Clinical Immunology, Referral centre for autoimmune cytopenia in adults (CeReCAI),Dijon University Hospital,DIJON,France
,
Thibault GHESQUIERE
Affiliations:
Department of Internal Medicine and Clinical Immunology, Referral centre for autoimmune cytopenia in adults (CeReCAI),Dijon University Hospital,DIJON,France
,
Marine THEBAULT
Affiliations:
Department of Internal Medicine and Clinical Immunology, Referral centre for autoimmune cytopenia in adults (CeReCAI),Dijon University Hospital,DIJON,France
,
François MAURIER
Affiliations:
Department of Internal Medicine,Groupe Hospitalier UNEOS,METZ,France
,
Thibault MAILLET
Affiliations:
Service de Médecine Interne - Unité 11,Centre Hospitalier de Mâcon, Groupe Hospitalier Bourgogne Méridionale,MACON,France
,
Maxime SAMSON
Affiliations:
Department of Internal Medicine and Clinical Immunology, Referral centre for autoimmune cytopenia in adults (CeReCAI),Dijon University Hospital,DIJON,France
,
Philippe SAAS
Affiliations:
UMR1098, RIGHT Interactions Greffon-Hôte-Tumeur/Ingénierie Cellulaire et Génique,Université Bourgogne Franche-Comté, INSERM, EFS BFC,DIJON,France
,
Bernard BONNOTTE
Affiliations:
Department of Internal Medicine and Clinical Immunology, Referral centre for autoimmune cytopenia in adults (CeReCAI),Dijon University Hospital,DIJON,France
Sylvain AUDIA
Affiliations:
Department of Internal Medicine and Clinical Immunology, Referral centre for autoimmune cytopenia in adults (CeReCAI),Dijon University Hospital,DIJON,France
EHA Library. Ciudad M. 06/09/21; 325457; EP697
Marion Ciudad
Marion Ciudad
Contributions
Abstract
Presentation during EHA2021: All e-poster presentations will be made available as of Friday, June 11, 2021 (09:00 CEST) and will be accessible for on-demand viewing until August 15, 2021 on the Virtual Congress platform.

Abstract: EP697

Type: E-Poster Presentation

Session title: Enzymopathies, membranopathies and other anemias

Background
Warm autoimmune hemolytic anemia (wAIHA) is a rare acquired autoimmune disease mediated by antibodies targeting red blood cells. Until now, the involvement of T helper (Th) lymphocytes has been scarcely studied in humans, most of the conclusions being extrapolated from animal models. Although still debated, a skewing to a pro-inflammatory Th17 polarization seems to be associated with AIHA. On the other hand, a quantitative regulatory T cell (Treg) deficiency has been shown in one study, but their suppressive functions were not assessed. Importantly, the balance between the pro- and anti-inflammatory T cell responses has never been measured simultaneously.

Aims

Assuming that an imbalance between the pro- and the anti-inflammatory T cell responses could participate to AHAI pathogenesis, we quantified the different circulating helper T cell (Th) subsets, Tregs and assessed their functional capabilities and their transcriptomic profiles.

Methods

Newly diagnosed wAIHA patients (n=20) were compared to sex- and age-matched healthy donors (n=30). Cells were quantified at baseline by flow cytometry, and polarization was determined by intracellular cytokine staining after stimulation with PMA and ionomycin. Treg functions were evaluated by their capability to inhibit effector T cell proliferation. Cytokines were measured by luminex assays in culture supernatants and sera. RNA sequencing was performed on Treg isolated from 4 patients and 4 controls by fluorescence-activated cell sorting (CD4+CD25HiCD127-).

Results

AHAI patients have a decrease in circulating Treg (CD4+CD25HiFoxp3+, 3.20% vs. 4.45%, p=0.008), mostly affecting activated Treg (CD4+Foxp3hiCD45RA-), known to arbor high suppressive capabilities. Moreover, Treg have a functional deficiency in AIHA, as shown by their lower ability to inhibit T cell proliferation (51% vs. 73%, p=0.03) associated to a trend to a lower inhibition of the secretion of pro-inflammatory cytokines (TNF-α, IFN-γ and IL-2) measured in coculture supernatants. Treg transcriptomic revealed an activated profile, as supported by an engagement in transcriptional and translational processes, an activation of TCR and IL-2 signaling, and an increased expression of CTLA-4 mRNA. Interestingly, TNF signaling pathway was also engaged, probably due to the increase in TNF-α in the sera of patients (2.29 vs. 1.24 pg/ml, p=0.002).


Although, Th polarization assessed by flow cytometry did not differ between patients and controls, the production of IL-17 by effector T cells measured in supernatants was higher during AHAI. Considering the Th17/Treg ratio, we observed an unbalance (0.023 vs. 0.01, p=0.01) of the pro-inflammatory over the anti-inflammatory T cell response during AIHA.

Conclusion

This work is the first to provide a concomitant evaluation of effector and regulatory T cells in human AIHA, showing an imbalance in the Th17/Treg ratio, associated with a quantitative and functional deficiency of Treg. Most interestingly, our results pointed out TNF-α as an interesting cytokine to target as its concentration is elevated in the sera and could participate to the inhibition of Treg functions.

Keyword(s): Autoimmune hemolytic anemia (AIHA), Regulatory T cell, T cell response

Presentation during EHA2021: All e-poster presentations will be made available as of Friday, June 11, 2021 (09:00 CEST) and will be accessible for on-demand viewing until August 15, 2021 on the Virtual Congress platform.

Abstract: EP697

Type: E-Poster Presentation

Session title: Enzymopathies, membranopathies and other anemias

Background
Warm autoimmune hemolytic anemia (wAIHA) is a rare acquired autoimmune disease mediated by antibodies targeting red blood cells. Until now, the involvement of T helper (Th) lymphocytes has been scarcely studied in humans, most of the conclusions being extrapolated from animal models. Although still debated, a skewing to a pro-inflammatory Th17 polarization seems to be associated with AIHA. On the other hand, a quantitative regulatory T cell (Treg) deficiency has been shown in one study, but their suppressive functions were not assessed. Importantly, the balance between the pro- and anti-inflammatory T cell responses has never been measured simultaneously.

Aims

Assuming that an imbalance between the pro- and the anti-inflammatory T cell responses could participate to AHAI pathogenesis, we quantified the different circulating helper T cell (Th) subsets, Tregs and assessed their functional capabilities and their transcriptomic profiles.

Methods

Newly diagnosed wAIHA patients (n=20) were compared to sex- and age-matched healthy donors (n=30). Cells were quantified at baseline by flow cytometry, and polarization was determined by intracellular cytokine staining after stimulation with PMA and ionomycin. Treg functions were evaluated by their capability to inhibit effector T cell proliferation. Cytokines were measured by luminex assays in culture supernatants and sera. RNA sequencing was performed on Treg isolated from 4 patients and 4 controls by fluorescence-activated cell sorting (CD4+CD25HiCD127-).

Results

AHAI patients have a decrease in circulating Treg (CD4+CD25HiFoxp3+, 3.20% vs. 4.45%, p=0.008), mostly affecting activated Treg (CD4+Foxp3hiCD45RA-), known to arbor high suppressive capabilities. Moreover, Treg have a functional deficiency in AIHA, as shown by their lower ability to inhibit T cell proliferation (51% vs. 73%, p=0.03) associated to a trend to a lower inhibition of the secretion of pro-inflammatory cytokines (TNF-α, IFN-γ and IL-2) measured in coculture supernatants. Treg transcriptomic revealed an activated profile, as supported by an engagement in transcriptional and translational processes, an activation of TCR and IL-2 signaling, and an increased expression of CTLA-4 mRNA. Interestingly, TNF signaling pathway was also engaged, probably due to the increase in TNF-α in the sera of patients (2.29 vs. 1.24 pg/ml, p=0.002).


Although, Th polarization assessed by flow cytometry did not differ between patients and controls, the production of IL-17 by effector T cells measured in supernatants was higher during AHAI. Considering the Th17/Treg ratio, we observed an unbalance (0.023 vs. 0.01, p=0.01) of the pro-inflammatory over the anti-inflammatory T cell response during AIHA.

Conclusion

This work is the first to provide a concomitant evaluation of effector and regulatory T cells in human AIHA, showing an imbalance in the Th17/Treg ratio, associated with a quantitative and functional deficiency of Treg. Most interestingly, our results pointed out TNF-α as an interesting cytokine to target as its concentration is elevated in the sera and could participate to the inhibition of Treg functions.

Keyword(s): Autoimmune hemolytic anemia (AIHA), Regulatory T cell, T cell response

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