Open Access Open Badges Research article

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

Marie-Quitterie Picat1234, Rodolphe Thiébaut1234*, François Lifermann5, Xavier Delbrel6, Daniel Adoue7, Linda Wittkop1234, Anne-Laure Fauchais8, Patrick Rispal9, Jean-François Moreau101112 and Jean-François Viallard1314*

Author Affiliations

1 INSERM, ISPED, Centre INSERM U897-Epidemiologie-Biostatistique, Bordeaux F-33076, France

2 University of Bordeaux, ISPED, Centre INSERM U897-Epidemiologie-Biostatistique, Bordeaux F-33076, France

3 Department of Medical Information, Bordeaux University Hospital, Bordeaux F-33000, France

4 INRIA SISTM, F-33405 Talence, France

5 Department of Internal Medicine, Dax Hospital, Dax F-40107, France

6 Department of Internal Medicine, Pau Hospital, Pau F-64046, France

7 Department of Internal Medicine, Purpan Hospital, Toulouse F-31059, France

8 Department of Internal Medicine, Dupuytren Hospital, Limoges F-87042, France

9 Department of Internal Medicine, Agen Hospital, Agen F-47923, France

10 University of Bordeaux, Bordeaux F-33076, France

11 CNRS, UMR 5164, Bordeaux F-33076, France

12 Bordeaux University Hospital, Laboratory of Immunology, Bordeaux F-33076, France

13 Department of Internal Medicine and Infectious Diseases, Bordeaux University Hospital, Pessac F-33604, France

14 Hôpital Haut-Lévêque - Service de médecine interne, 5, avenue de Magellan, Pessac 33604, France

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BMC Immunology 2014, 15:13  doi:10.1186/1471-2172-15-13

Published: 12 March 2014



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.


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.


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.

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