Common Variable Immunodeficiency

immunoloji-17-1-2024

Eray YILDIZa
aKahramanmaraş Necip Fazıl City Hospital, Clinic of Immunology and Allergy Diseases, Kahramanmaraş, Türkiye

Yıldız E. Common variable immunodeficiency. Çölkesen F, ed. Primary Immunodeficiency Diseases in Adults. 1st ed. Ankara: Türkiye Klinikleri; 2024. p.16-27.

ABSTRACT
Common Variable Immunodeficiency (CVID) is an inborn error of immunity, characterised by defective immunoglobulin (Ig) production associated with impaired B-cell differentiation. CVID is the most common symptomatic immunodeficiency in adults. It is seen together with comorbidities and heterogenous complications such as recurrent bacterial infections, autoimmune disorders, gastrointestinal diseases, lymphoproliferation, chronic lung disease, and increased risk of malignancy. The diagnosis of CVID is based on decreased serum IgG levels with decreased serum IgM and/or IgA levels, poor response to vaccines and/or absence of isohemagglutinins, and exclusion of other causes of hypogammaglobulinemia. Immune phenotyping of peripheral lymphocytes and molecular analysis are diagnostically helpful but not essential. CVID patients are currently treated only symptomatically. The most important approaches in treatment are immunoglobulin replacement therapy and prophylactic antibiotic use.

Keywords: Primary immune deficiency; immunophenotyping; immunoglobulin replacement therapy; common variable immunodeficiency

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