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J Allergy Clin Immunol Pract. 2016 Nov - Dec;4(6):1147-1157. doi: 10.1016/j.jaip.2016.07.002. Epub 2016 Aug 10.

Exclusion of Patients with a Severe T-Cell Defect Improves the Definition of Common Variable Immunodeficiency.

Author information

1
Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), Paris, France.
2
Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), Paris, France; EA3518, Université Paris Diderot Paris 7, Paris, France.
3
Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), Paris, France; Inserm U1126, Centre Hayem, Hôpital Saint-Louis, Paris, France.
4
Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), Paris, France; EA3518, Université Paris Diderot Paris 7, Paris, France. Electronic address: eric.oksenhendler@aphp.fr.

Abstract

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.

KEYWORDS:

CVID; Definition; Hypogammaglobulinemia; Immunodeficiency; Late onset combined immunodeficiency; Prognosis

PMID:
27522107
DOI:
10.1016/j.jaip.2016.07.002
[Indexed for MEDLINE]

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