Exclusion of Patients with a Severe T-Cell Defect Improves the Definition of Common Variable Immunodeficiency
| العنوان: | Exclusion of Patients with a Severe T-Cell Defect Improves the Definition of Common Variable Immunodeficiency |
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| المؤلفون: | Rémi Bertinchamp, Laurence Gérard, David Boutboul, Marion Malphettes, Claire Fieschi, Eric Oksenhendler, E. Oksenhendler, C. Fieschi, M. Malphettes, L. Galicier, D. Boutboul, J.P. Fermand, J.F. Viallard, A. Jaccard, C. Hoarau, Y. Lebranchu, A. Bérezné, L. Mouthon, M. Karmochkine, N. Schleinitz, I. Durieu, R. Nove-Josserand, V. Chanet, V. Le-Moing, N. Just, C. Salanoubat, R. Jaussaud, F. Suarez, O. Hermine, P. Solal-Celigny, E. Hachulla, L. Sanhes, M. Gardembas, I. Pellier, P. Tisserant, M. Pavic, B. Bonnotte, J. Haroche, Z. Amoura, L. Alric, M.F. Thiercelin, L. Tetu, D. Adoue, P. Bordigoni, T. Perpoint, P. Sève, P. Rohrlich, J.L. Pasquali, A.S. Korganow, P. Soulas, L.J. Couderc, E. Catherinot, P. Giraud, A. Baruchel, I. Deleveau, F. Chaix, J. Donadieu, F. Tron, C. Larroche, A.P. Blanc, A. Masseau, M. Hamidou, G. Kanny, M. Morisset, F. Millot, O. Fain, R. Borie, A. Perlat, B. Bienvenue, B. Autran, G. Gorochov, J.L. Garnier, H. Moins, G. Maki, L. Gérard |
| المساهمون: | Service d'Immunopathologie [Hôpital Saint-Louis, Paris], Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Recherche clinique appliquée à l'hématologie ((EA_3518)), Université Paris Diderot - Paris 7 (UPD7), Différenciation et progression tumorale des lymphocytes, École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut Universitaire d'Hématologie (IUH), Université Paris Diderot - Paris 7 (UPD7)-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris Diderot - Paris 7 (UPD7)-CHU Saint Louis [APHP], École pratique des hautes études (EPHE)-Institut Universitaire d'Hématologie (IUH) |
| المصدر: | Journal of Allergy and Clinical Immunology: In Practice Journal of Allergy and Clinical Immunology: In Practice, Elsevier, 2016, 4 (6), pp.1147-1157. ⟨10.1016/j.jaip.2016.07.002⟩ |
| سنة النشر: | 2016 |
| مصطلحات موضوعية: | 0301 basic medicine, Adult, CD4-Positive T-Lymphocytes, Male, Pediatrics, medicine.medical_specialty, Opportunistic infection, T cell, Hypogammaglobulinemia, Population, Late onset combined immunodeficiency, Opportunistic Infections, 03 medical and health sciences, Agammaglobulinemia, Overall survival, Immunology and Allergy, Medicine, Immunodeficiency, Humans, [SDV.IMM.ALL]Life Sciences [q-bio]/Immunology/Allergology, education, education.field_of_study, business.industry, Common variable immunodeficiency, CVID, Definition, Middle Aged, medicine.disease, Prognosis, Confidence interval, 3. Good health, Surgery, CD4 Lymphocyte Count, 030104 developmental biology, medicine.anatomical_structure, Common Variable Immunodeficiency, Female, business |
| الوصف: | International audience; BACKGROUND:In 2014, the European Society for Immune Deficiencies (ESID) revised the common variable immunodeficiency (CVID) diagnosis criteria by incorporating new clinical and biological markers. The new definition appeared more restrictive but had not yet been evaluated in a large cohort of patients.OBJECTIVE:The objective of this study was to evaluate the impact of this new definition in a large cohort of patients with primary hypogammaglobulinemia.METHODS:Evaluation of 3 different CVID definitions (ESID/Pan-American Group for Immunodeficiency [PAGID] 1999, ESID 2014, DEFI 2015) in 521 patients included in the French DEFI study with a diagnosis of primary hypogammaglobulinemia.RESULTS:Using the ESID/PAGID 1999 definition, 351 patients were classified as CVID. The new ESID 2014 definition excluded 62 (18%) patients. Most of them (n = 56; 90%) had a less severe disease, whereas 6 (10%) presented with a severe disease with major T-cell defect. We propose different criteria (occurrence of opportunistic infection or very low naive CD4+ T-cell count) to define this population with severe T-cell defect. Sixty-two patients fulfilled these criteria, represented 20% of the initial CVID population but accounted for 77% of the deaths, with a 5-year overall survival of 67.6% (95% confidence interval, 51.0-79.6), and were considered as late onset combined immunodeficiency (LOCID).CONCLUSIONS:The new ESID definition for CVID still fails to exclude a large number of patients with severe T-cell defect. We propose a new definition (DEFI 2015) that excluded more patients with a T-cell defect and consider these patients as LOCID. This population has a poor outcome and should be considered as a distinct group requiring specific care. |
| تدمد: | 2213-2201 2213-2198 |
| DOI: | 10.1016/j.jaip.2016.07.002⟩ |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3eb10a53fac240dd53b1a320b55a3023 https://pubmed.ncbi.nlm.nih.gov/27836061 |
| Rights: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....3eb10a53fac240dd53b1a320b55a3023 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 22132201 22132198 |
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| DOI: | 10.1016/j.jaip.2016.07.002⟩ |