86. Primary immunodeficiency syndromes: definition, classification, causes, changes in the organs of the immune system, complications.

Immunodeficiency syndromes are an extreme manifestation of the insufficiency of the immune system.

Primary-underdevelopment (hypoplasia, aplasia) of the immune system-congenital, hereditary

1. Primary immunodeficiency may be associated with insufficiency:

1. cellular and humoral immunity (combined)

2. cellular immunity

3. humoral immunity

1)Combined immunodeficiency syndromes -

a)(Combined Glanzmann and Rinicker type)agammaglobulinemia of the Swiss type.

The type of inheritance is autosomal recessive

Clinical and morphological manifestations are Hypoplasia of the thymus and peripheral.lymphoid tissue, lymphopenia, frequent infectious diseases.

Immune disorders are a total defect of cellular and humoral immunity, loss of the ability to synthesize Ig.

b)Ataxia-Louis Bar telangiectasia

The type of inheritance is autosomal recessive

Clinical and morphological manifestations are Hypoplasia of the thymus gland and peripheral.lymphoid tissue,lymphopenia,atrophy of the cerebellar cortex (ataxia),telangiectasia of the bulbous conjunctiva,mesenchyma.The evil one.tumors, recurrent pneumonia

Immune disorders-cellular immunity defect, Ig deficiency, frequent IgA deficiency

c)Combined type with the presence of B lymphocytes and immunoglobulins (Nezelof syndrome)

Type of inheritance-

Clinical and morphological manifestations - hypoplasia of the thymus.glands and peripherals.lymphoid tissue, lymphopenia, sepsis.

Immune disorders - the content of immunoglobulins.normal, dysgammaglobulinemia is possible.A defect in cellular immunity.

2) Cellular immunity deficiency syndrome

a)Agenesis or hypoplasia of the thymus gland (Digeorge syndrome)

The type of heredity is not known

Clinical and morphological manifestations are the absence of the thymus gland and parathyroid glands (tetany), the absence of T-lymphocytes.

Immune disorders-the content of immunoglobulins is normal.A defect in cellular immunity.

3) The syndrome of insufficient humoral immunity.

a)Agammaaglobulinemia linked to the X chromosome (Bruton syndrome)

The type of heredity is linked to the X chromosome

Clinical and morphological-the thymus gland is preserved.The absence of B-dependent zones of plasmocyte cells.a number of lymph nodes and spleen, private infectious diseases.

Immune disorders are a defect in humoral immunity, loss of the ability to synthesize immunoglobulins.

b)Selective IgA deficiency (West syndrome)

The type of heredity is linked to the X chromosome

Clinical and morphological manifestations - the structure of lymphoid tissue is preserved.Manifestations of allergies.Frequent infections of the respiratory tract and gastrointestinal tract in combination with autoimmune diseases, malabsorption syndrome, sometimes with tumors.

Immune disorders-loss of the ability to synthesize Ig A.

1. Secondary immunodeficiency syndromes (acquired)-in connection with the disease or the type of treatment

-AIDS-acquired immunodeficiency syndrome -self-standing.disease

Other infections lead to the development of secondary immunodeficiency.leukemia, cancer.lymphomas, thymoma, sarcoidosis. It is based on a defect in humoral and cellular immunity as a result of a defect in the population of B and T lymphocytes

Treatment leading to secondary immunodeficiency:radiation therapy.taking corticosteroids.immunosuppressants, anti-lymphocytic serum, thymectomy.drainage of the thoracic duct.