Single-center report of antibody deficiencies in Azerbaijan

Abstract

Abstract The most common type of Primary Immune Deficiencies (PID) are Antibody Deficiencies (AD) characterized by the recurrent bacterial infections. Usually the immune disbalance is observed as a decrease in B-cell function, hypogammaglobulinemia and deficiency of IgG and IgA or all three IgG, IgA and IgM antibodies. Early onset acute or chronic infections in gastrointestinal, respiratory and urinary tract are the most common symptoms of AD. The aim of the study was to analyze the data of 126 pediatric and adult patients diagnosed as AD in our PID center between 2010–2019. Immune status of the patients was examined by phenotyping of immune cells in peripheral blood, measurement of serum IgM, IgG, IgA and IgE levels, evaluation of phagocytic activity by NBT, and detection of CIC by photometric method. Additional abdominal CT and ultrasound examinations were also performed. From 126 pediatric and adult patients registered in PID Center, Baku, Azerbaijan, 9 patients were diagnosed with sIgA deficiency, 26 were common variable immune deficiency, 9-Bruton disease and 2 patients with Hyper IgM syndrome. Patient cohort included 5 adults and 41 between 12 months to 11 years age. At the time of the study, 37 patients were receiving intravenous immune globulin (IVIG) as a replacement therapy (31 patients were on regular IVIG). IVIG therapy was associated with decreased incidence of infections and overall improvement in well-being of the patients. It is important to consider hypogammaglobulinemia in patients with a history of recurrent and severe infections. We recommend that patients’ immune status should be examined including evaluation of serum immunoglobulin levels and B and T-cell subsets.