Bruton agammaglobulinemia is a hereditary (X-linked) immunodeficiency disorder characterized by the absence of mature B-cells, which leads to severe antibody deficiency and recurrent infections. Clinical manifestations occur as soon as the protective effect of maternal immunoglobulins decreases, approximately at the age of six months. The first sign is frequent respiratory infections, which can have serious progression.
Aim: evaluating the spectrum of infections in Bruton agammaglobulinemia
Were reviewed medical records of 4 patients with Bruton agammaglobulinemia focusing on immunoglobulins, phenotyped peripheral blood lymphocytes and infections.
In all children the first sign of alarm was the frequent early-onset infections. In 2 of the children were present purulent infections with different localizations (meningitis, empyema, sinusitis, osteomyelitis) with progressive severe progression. Recurrent respiratory infections over time have complicated with chronic bronchopulmonary phenomena with bronchial deformities, pulmonary fibrosis, bronchiectasis. The immunological investigation in all patients indicated a reduction of serum immunoglobulin values: IgA - 1.78±0.9 mg/dl; IgM - 1.8±0.67 mg/dl; IgG - 6.75±0.54 mg/dl. Determination of lymphocyte populations in these patients resulted with normal T – 49±13.8% lymphocyte variation and a significant reduction in B cells of 8.8±3.03%.
Lack of antibodies to agammaglobulinemia Bruton leads to a polymorphism of infections with different localization, the respiratory system being predominantly affected.