T-cell activation discriminates subclasses of symptomatic primary humoral immunodeficiency diseases in adults.

BACKGROUND: Symptomatic Primary Humoral Immunodeficiency Diseases (PHID) constitute a highly heterogeneous group of diseases characterized by a shared hypogammaglobulinemia, resulting in increased risk of recurrent or severe infections. Associations have been described with a variety of immunological abnormalities involving B and T-cell differentiation, T-cell activation and innate immunity. However, PHID discrimination remains based on B-lymphocyte abnormalities and other components of the immune system have not been sufficiently taken into account. We carried out unsupervised and supervised methods for classification in a cohort of 81 symptomatic PHID patients to evaluate the relative importance of 23 immunological parameters and to select relevant markers that may be useful for diagnosis and prognosis. RESULTS: We identified five groups of patients, among which the percentage of PHID complications varied substantially. Combining the set of markers involved in PHID supported the existence of two distinct mechanisms associated with complications. Switched memory B-cell attrition and CD8+ HLA-DR + activated T-cell increase were the prominent abnormalities observed in PHID complications. Furthermore, in a subgroup of 57 patients with common variable immunodeficiency, the classification that added CD8+ HLA-DR + to the consensual EUROclass classification was better than the EUROclass model in predicting complications. CONCLUSION: These results highlight the importance of T-cell activation that may improve discrimination of PHID patients in specific subgroups and help to identify patients with different clinical outcomes.

Mots clés

Symptomatic primary humoral immunodeficiency T-cell activation HLA-DR marker Hierarchical clustering Principal component analysis Common variable immunodeficiency IgG subclass deficiency Good ' s syndrome

Domaines

Immunologie

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https://inserm.hal.science/inserm-00981320

Soumis le : mardi 22 avril 2014-05:36:48

Dernière modification le : mercredi 17 janvier 2024-09:52:06

Archivage à long terme le : lundi 10 avril 2017-16:14:33

Dates et versions

inserm-00981320 , version 1 (22-04-2014)

Identifiants

HAL Id : inserm-00981320 , version 1
DOI : 10.1186/1471-2172-15-13
PUBMED : 24621280

Citer

Marie-Quitterie Picat, Rodolphe Thiébaut, François Lifermann, Xavier Delbrel, Daniel Adoue, et al.. T-cell activation discriminates subclasses of symptomatic primary humoral immunodeficiency diseases in adults.. BMC Immunology, 2014, 15 (1), pp.13. ⟨10.1186/1471-2172-15-13⟩. ⟨inserm-00981320⟩

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