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Common variable immunodeficiency (CVI): case report

Introduction

The CVI is the second most common primary immunodeficiency. The prevalence of CIV in the world is approximately 1:25,000. The age of presentation of ICV has peaks in the first decade if life and the beginning of the third decade. Both sexes are affected. Diagnostic criteria according to ESID/PAGID are: - recurrent infections; -age more 4 years; -reduced levels of IgG; decrease of IgA and/or IgM; exclusion of the other causes of hypogammaglobulinemia; - no isohemaglutininas and response to the vaccine. Five clinical phenotypes are described: only infections, autoimmunity, polyclonal lymphocytic infiltration, malignancy and enteropathy. The treatament of patients with CVI is performed with the use of an infusion of immunoglobulin.

Objective

To report a patient with CVI associated with infections/ infestations recurrent and splenomegaly. Case description: H.S.M.A, male, 8 years old, eight pneumonia, low weight gain, infestation by Giardia lamblia and splenomegaly (physical examination and ultrasound).

Tests

HIV ELISA negative. IgG=614mg/dl; IgM=19mg/dl; IgA=24mg/dl; CD4=24 (742mm3); CD8=70% (2150/mm3) and CD19=247/mm3. Lymphocyte B total=9.2%.

Discussion

The CVI can be associated with some phenotypes, such as the occurrence of infections/infestations recurrent and splenomegaly. The levels of immunoglobulins are below the 3 percentile for age, the percentage of b lymphocytes in the minimum threstold, CD8 high.

Conclusion

Reiterate the importance of the diagnosis of primary immunodeficiency in patients with infections/infestations recurrent splenomegaly and in this case the CVI by important prevalence.

Consent

Written informed consent was obtained from the patient for publication of this abstract and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.

References

  1. Conley ME, Notarangelo LD, Etzioni A: Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clinical immunology. 1999, 93 (3): 190-7. 10.1006/clim.1999.4799.

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This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Gomes, L.C.B. Common variable immunodeficiency (CVI): case report. World Allergy Organ J 8 (Suppl 1), A268 (2015). https://doi.org/10.1186/1939-4551-8-S1-A268

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  • DOI: https://doi.org/10.1186/1939-4551-8-S1-A268