Agammaglobulinemia Alymphocytic

Alimphocytic agammaglobulinemia is another name for lymphopenic agammaglobulinemia.

Lymphopenic agammaglobulinemia is a rare autosomal recessive disease characterized by the absence or sharp decrease in the level of immunoglobulins in the blood. The cause of the disease is mutations in the genes responsible for the maturation of B lymphocytes. This leads to impaired antibody production and severe immunodeficiency.

The main symptoms of lymphopenic agammaglobulinemia: frequent bacterial infections of the respiratory tract and gastrointestinal tract, delayed physical and psychomotor development. Diagnosis is based on analysis of the level of immunoglobulins and the number of lymphocytes in the blood. Treatment consists of lifelong replacement therapy with intravenous immunoglobulins. The prognosis with timely diagnosis and adequate therapy is favorable.