Congenital deficiency of cellular and humoral immunity


  • Lack of humoral immunity
  • Cell-mediated immunity
  • Combined deficiency of humoral and cellular immunity

  • Lack of humoral immunity

    Lack of humoral immunity50-60% of primary immunodeficiencies and manifests violation antibody production (serum proteins, which are produced in the human body in response to ingress of foreign agents - viruses, bacteria). This group includes the isolated immunoglobulin A deficiency, isolated deficiency of immunoglobulins of other classes, the lack of immunoglobulins of several classes. Possible failure of humoral immunity in normal concentrations of antibodies. This is caused by decrease in the level of antibodies to a specific group of antigens, such as lower levels of antibodies to bacterial carbohydrate antigens.

    Isolated deficiency of immunoglobulin A

    Isolated deficiency of immunoglobulin AIt is the most common. It occurs as a result of the defect terminal differentiation of cells secreting the immunoglobulin A. Most patients with immunoglobulin A deficiency are asymptomatic. Only a small number of patients are prone to occurrence of lung and intestinal infections.

    Cell-mediated immunity

    Cell-mediated immunity is5-10% of primary immunodeficiencies and manifests violation proliferation (proliferation) and differentiation of T-lymphocytes or stem cell deficiency. Primary violation of cellular immunity in most cases accompanied by a secondary violation of the synthesis of antibodies.

    Lack of education of T cell immunity

    Lack of T-cells leads to increasedthe body's sensitivity to viruses and fungi, which is based on the destruction of the cellular immune response. DiDzhorzhi and Nezelofa syndrome syndrome caused by underdeveloped thymus during fetal development. These patients can not detect the reactions of cellular immunity, and although their bodies produce antibodies, antibody response is weakened.

    Immune deficiency of B-cells

    Lack of B lymphocytes leads tocongenital agammaglobulinemia Bruton, found only in men. Boys exposed to repeated infections, which are caused by pyogenic bacteria - staphylococci and streptococci. The disease is caused by a recessive gene, localized on the X chromosome, and is detected only in boys, having a set of sex chromosomes XY. In this form no immunodeficiency B cells, no reproduction centers of lymphoid tissues, resulting in the serum fails to detect immunoglobulins. At the same time patients have normal thymic development. T cells are quantitatively and functionally no different from healthy children.

    Reducing the content of gammaglobulin

    Hypogammaglobulinemia (decrease in the numbergammaglobulin) is also characterized by a purulent infection. Lack of immunoglobulins found in severely premature babies, when the level falls immunoglobulins derived from the mother.

    Combined deficiency of humoral and cellular immunity

    Congenital deficiency of cellular and humoral immunityCombined deficiency of humoral andcellular immunity is 20-25% of all primary immunodeficiencies. This group includes diseases caused by a primary violation of proliferation (proliferation) and differentiation of B-lymphocytes and T-lymphocytes. Reducing the number of T-lymphocyte and immunoglobulin levels in blood it is most pronounced in severe combined immunodeficiency. This is one of the most severe forms of congenital immunodeficiency. Characterized by the occurrence of various infections (viral, fungal, bacterial) disease shortly after birth, which leads to early death (usually in the first year of life).

    Combined deficiency of humoral andcellular immunity is often accompanied by other congenital diseases, such as thrombocytopenia with Wiskott-Aldrich syndrome, or congenital heart disease and hypocalcaemia (low calcium in the blood) with DiGeorge syndrome.

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