eISSN: 1644-4124
ISSN: 1426-3912
Central European Journal of Immunology
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2/2019
vol. 44
 
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abstract:
Clinical immunology

Characteristics of the patients followed with the diagnosis of common variable immunodeficiency and the complications

Semiha Bahceci Erdem
,
Nesrin Gulez
,
Ferah Genel
,
Sait Karaman
,
Hikmet T. Nacaroglu

(Centr Eur J Immunol 2019; 44 (2): 119-126)
Online publish date: 2019/07/30
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Introduction
In this study, we aimed to retrospectively evaluate the clinical and laboratory findings and complications of 28 common variable immunodeficiency (CVID) patients.

Material and methods
The clinical features and laboratory data of 28 CVID patients were evaluated.

Results
Nineteen patients were male. In 53.5% of the cases, complications included inflammatory bowel disease, cytopenia, bronchiectasis, granulomatous lymphocytic interstitial lung disease (ILD) and asthma. In their immunological evaluations, IgG, IgM, and IgA mean values were 474.8 ±214.1 mg/dl; 56.7 ±41.9 mg/dl; 35.3 ±58.2 mg/dl, respectively, and the vaccine response was positive in 64.2% of the cases. In all age groups, absolute lymphocyte counts, naive (CD19+IgD+27-), nonswitch (CD19+IgD-27+) memory B cells were numerically higher when compared to the data of healthy children; however, although switch memory (CD19+IgD+27+) B cells were proportionally low in the 4-8 and 12-18 age groups, they were low both numerically and proportionally in the 8-12 age group. No statistically significant difference was found between the cases with complications and without complications. But the cases with pulmonary complications were compared within the group, the CD8 ratio was high but the IgA level was low in patients with bronchiectasis and CD3 was numerically and proportionally low in the cases with ILD compared to others. According to the Paris classification, 11/27 (40.7%) of the cases, 3/27 (11.1%) of them and 13/27 (48.2%) of them were evaluated as MB0, MB1, and MB2, respectively.

Conclusions
In genetic studies, TACI (trans-membrane activator and calcium-modulating cyclophilin ligand interactor – TNFRSF13B) mutation was found positive in 25% of the cases.

keywords:

primary immunodeficiency, hypogammaglobulinemia, common variable immunodeficiency, TACI

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