Textbook - BIOLOGICAL CHEMISTRY - Hubskyi Y.I. - 2000

Chapter VI. BIOCHEMISTRY OF PHYSIOLOGICAL FUNCTIONS AND SPECIALIZED TISSUES

CHAPTER 30. BIOCHEMISTRY OF IMMUNE PROCESSES

30.5. BIOCHEMICAL MECHANISMS OF IMMUNODEFICIENCY STATES

Disorders in the functioning of the human immune system—immunodeficiency states—develop under conditions of damage to specific links of cellular or humoral Immunity. According to their origin mechanism, they are classified into primary and secondary immunodeficiencies.

Primary immunodeficiencies are pathological conditions that occur due to the hereditary inability of The Human Body to respond to antigenic stimulation with the synthesis of appropriate classes of Antibodies (IMMUNOGLOBULINS) or The formation of a cellular Immune Response. They develop As a result of molecular defects in specific Regions of the genome responsible for the phenotypic manifestations of the T- and B-immune systems. The first manifestations of primary immunodeficiencies are observed in early childhood.

There are four classes of primary immunodeficiencies, which characterize the impairment of specific major Components of the immune system:

1. B-Cell deficiency (antibody deficiency).

2. T-cell deficiency.

3. Pathology of phagocytic Cells.

4. Pathology of The Complement System.

Let us consider Examples of some of the most common human primary immunodeficiencies.

Bruton's disease is X-linked agammaglobulinemia. This immunodeficiency is characterized by a drastic decrease in antibacterial immunity activity, which manifests as severe bacterial infections. The Blood serum of patients shows a significant reduction in the concentration of IgG (approximately 10-fold compared to the norm), IgA and IgM (approximately 100-fold), as well as an absence of B-lymphocytes and plasma cells.

Swiss-type agammaglobulinemia is a disease characterized by a deficiency of both cellular (T-lymphocytes) and humoral (B-lymphocytes) immunity. The immunodeficiency is inherited in an autosomal recessive manner, manifesting predominantly in males.

Dysimmunoglobulinemias are a group of primary immunodeficiencies characterized by various patterns of disorders in the Synthesis and Secretion of individual classes of immunoglobulins (mostly IgG, IgM, and IgA).

Louis-Bar syndrome (Ataxia-telangiectasia) is an immunological deficiency manifested by neurological disorders (cerebellar-type ataxia) and the pathological dilation of Blood Vessels in the conjunctiva and Skin (telangiectasias). The blood of patients shows reduced cellular immunity response activity, an absence of IgA, and low levels of IgG.

DiGeorge syndrome is congenital thymic hypoplasia; the disease is characterized by Impairment of the T-immune system in childhood.

Secondary immunodeficiencies are pathological conditions that develop as a result of damage to specific links of cellular or humoral immunity by pathogenic factors of biological, chemical, or physical origin. Most commonly, they develop under METABOLISM/18.html">The Influence of lymphotropic viral infection (AIDS, or Acquired Immunodeficiency Syndrome in humans), toxic factors, and ionizing radiation.