LECTURE No. 1. Immunodeficiency conditions in children. Clinic, diagnosis, treatment

Immunity is a way to ensure and maintain antigenic homeostasis.

Immunodeficiencies - a decrease in the functional activity of the main components of the immune system, leading to a violation of the antigenic homeostasis of the body and, above all, to a decrease in the body's ability to defend itself against microbes, manifested in an increased infectious morbidity.

Classification of immunodeficiency states:

1) primary immunodeficiencies;

2) secondary immunodeficiencies.

1. Primary immunodeficiencies

Primary immunodeficiencies are genetically determined monogenic diseases transmitted as an autosomal recessive or X-linked trait. There is also an autosomal dominant type of inheritance.

Primary immunodeficiencies are congenital disorders of the immune system with defects in one or more of its components (cellular or humoral immunity, phagocytosis, complement system).

Classification of primary immunodeficiency states:

1) pathology of the humoral link of immunity, i.e., insufficiency in the production of antibodies;

2) pathology of the cellular link of immunity mediated by T-lymphocytes;

3) combined forms (SCID) of humoral and lymphocytic deficiency.

Clinical picture. The clinical picture of immunodeficiency states has common features.

1. Recurrent and chronic infections of the respiratory tract, paranasal sinuses, skin, mucous membranes, gastrointestinal tract, caused by opportunistic infections, protozoa, fungi, tending to generalize, septicemia and torpid to the usual reaction.

2. Hematological deficits: leukocytopenia, thrombocytopenia, anemia.

3. Autoimmune disorders: arthritis, scleroderma, chronic active hepatitis, thyroiditis.

4. Sometimes IDS are combined with allergic reactions in the form of eczema, Quincke's edema.

5. Tumors and lymphoproliferative diseases are more common in IDS.

6. Often IDS are combined with malformations.

7. Patients with IDS have digestive disorders, diarrheal syndrome, malabsorption syndrome.

8. Patients with IDS have unusual reactions to vaccination.

9. Reticular dysgenesis (defect of maturation of lymphoid and myeloid cells).

10. Swiss type SCID (T- and B-lymphocytosis with a-globulinemia).

11. Omenn's syndrome (SCID with eosinophilia and T-cell infiltration of a number of organs).

12. X-linked SCID (low block of T-cell maturation in combination with normal B-cell differentiation).

13. Autosomal recessive SCID.

Severe combined immune deficiency manifests itself in the 1st month of life: symptoms of a severe multi-organ infectious process with malnutrition. On the part of the respiratory system - bronchopulmonary lesion. From the gastrointestinal tract - diarrhea.

Candidiasis. Thymic dysplasia. Immunologically, a defect in stem cell differentiation with profound immunity disorders (immunological silence syndrome) is assumed. Laboratory examination revealed lymphopenia (especially T-lymphocytes), a decrease and disturbance in the ratio of components in the T- and B-systems. Death occurs in the 1st month or 1-2 years of life.

Syndrome of reticular dyskinesia. Clinically - the death of the fetus or child immediately after birth. Immunologically - a defect in the maturation of all lymphoid and myeloid cells in the bone marrow.

"Swiss type" Clinical manifestations - from the 1st month of life, delayed weight gain, candidiasis, prolonged recurrent pneumonia, rashes. Hypoplasia of the thymus and lymph nodes. Immunologically - T- and B-alymphocytosis with agammaglobulinemia (with the exception of IgG in infants). Laboratory testing revealed a decrease in adenosine deaminase levels. Death occurs before the age of 2 years.

Louis-Barr syndrome (ataxia-telangiectasia). Clinical manifestations are characterized by ataxia. Often misdiagnosed as cerebral palsy. In the future - repeated infections of the respiratory system, sinusitis, skin and eye telangiectasia. Hypoplasia of the thymus, cerebellar degeneration. Immunologically - deficiency of T-lymphocytes (especially T-helpers) and immunoglobulins (especially IgA and IgE). Death of a patient under the age of 2 years.

Wiskott-Aldrich syndrome. Clinical manifestations: boys have frequent infections, hemorrhages, eczema, and neoplasms from birth. Immunologically - T-lymphocyte deficiency, isolated IgM deficiency. Laboratory tests revealed thrombocytopenia (maybe isolated). Severity and prognosis are variable (death may occur up to 10 years).

DiGeorge syndrome. Clinical manifestations in the form of prolonged and inflammatory processes in the lungs, dermatitis, heart and vascular defects, hypoparathyroidism (tetany, hypocalcemia, convulsions). Absence or hypoplasia of the thymus. Immunologically - a defect in the development of T cells at the level of T-lymphocyte precursors. In a laboratory study, a decrease in the level of immunoglobulins with a normal or increased number of B cells. The severity of immunodeficiency usually decreases over time.

Nezelof's syndrome. Clinical manifestations - from birth, purulent infections, sepsis. Atrophy of the thymus and lymph nodes. Immunologically - a sharp decrease in the level of B-lymphocytes, with a normal content of Ig in the blood. Death in the first months of life.

X-linked agammaglobulinemia (Bruton's disease). Clinical manifestations are characterized by a predisposition to purulent infections (increased susceptibility to echo and enteroviruses - vaccine polio and chronic echovirus infection). Hypoplasia of lymphoid tissue (absence of tonsils, small lymph nodes). Immunologically - the T-link is preserved, the block is at the level of formation of early B-lymphocytes. Laboratory tests revealed a sufficient number of B-cell precursors in the bone marrow and agammaglobulinemia in the blood. Males with hypogammaglobulinemia should be evaluated for the presence of a mutation in the bruton-tyrosine kinase gene.

Common variable immune deficiency. Clinically, XLA is more severe, there are signs of combined immunodeficiency, and oncological complications. Pyogenic infections, giardiasis, mycobacteriosis, gastrointestinal disorders; autoimmune disorders, including hematological ones. Immunologically - low Ig levels, the number of B cells is normal or reduced. As it progresses, B cells may disappear from the peripheral blood. The most common types are selective hyper-IgM syndrome (type I) and IgA deficiency (type IV). Hyper-IgM syndrome has at least two forms - X-linked and autosomal recessive.

Diagnostics. Diagnosis of primary immunodeficiency states is as follows:

1) selection of children at risk of primary CHD (attention must be paid):

a) the pedigree of the child, indicating in it cases of death of children in the family at an early age from inflammatory diseases;

b) the development of vaccination, repeated, chronic, multifocal and unusually ongoing infections, parasitic and fungal diseases;

c) the presence in the pedigree of autoimmune, allergic and tumor processes, hemopathy and pathology associated with sex;

d) the presence of associated syndromes (lagging behind in physical development, endocrinopathy, skin and neurological manifestations, etc.);

2) when assessing the immune system of children, it is necessary to take into account:

a) negative Mantoux tests after vaccination and BCG revaccination;

b) thymus dysplasia in young and middle-aged children;

c) the absence of an increase in regional lymph nodes in response to the inflammatory process;

d) hypoplasia of the tonsils or, on the contrary, a pronounced hypertrophy of the tonsil tissue and lymph nodes in combination with recurrent inflammatory processes;

3) assessment of routine laboratory tests:

a) detection in a clinical blood test: hemolytic or hypoplastic anemia, neutropenia, thrombocytopenia, absolute lymphopenia (less than 1000 in 1 mm3), absence of plasma cells in response to an acute infection - all this may indicate a syndrome of insufficiency in T- and B -system;

b) analysis of the proteinogram - detection of hypoproteinemia and hypoalbuminemia, low levels of B - and especially g-globulins - makes it possible to roughly but reliably judge the state of some immune functions;

4) identification of bright clinical non-immunological markers:

a) ataxia and bulbar telangiectasias - with Louis-Barr syndrome;

b) malformations of the main vessels and convulsions against the background of hypocalcemia - with Di-George's syndrome.

Treatment. Principles of therapy for primary immunodeficiency states:

1) hospitalization for in-depth immunological and molecular studies and the choice of the method of therapy;

2) adequate replacement immunotherapy, which allows many patients to lead a normal life;

3) bone marrow transplantation - a radical and almost routine treatment for many forms of IDS;

4) refusal to consider a patient with congenital IDS as therapeutically unpromising.

Scheme of modern therapy of primary IDS.

1. Infection control - in some cases, lifelong antibacterial and antifungal therapy.

2. Replacement immunotherapy with drugs containing antibodies: native plasma (cryopreserved or fresh); immunoglobulins for enteral (CIP - a complex immunoglobulin preparation for oral administration, containing 50% Ig G and 25% each - Ig M and Ig A), intramuscularly (IGVM) and intravenously (IGVI) administration. IVIG approved for use in the Russian Federation (intraglobin F, intraglobin human normal, Biaven V.I., vigam-liquid, vigam-S, octagam, sandoglobulin, pentaglobin). Doses for IDS: 100-400 mg / kg (5% solution 2-8 ml / kg) for 1 injection 1 time per day, 1 time in 1-4 weeks.

Clinical indications for the choice of IVIG: primary and secondary IDS, immunopathological diseases (thrombocytopenic purpura, Kawasaki disease). Sepsis and severe bacterial infections. Prevention of infections in IDS.

3. Replacement therapy by other means: in case of deficiency of adenosine deaminase - injections of polyethylene glycol; with a deficiency of the C1 inhibitor - the introduction of recombinant C1 ING.

4. Activation of the T- and B-systems of immunity - is effective only in secondary IDS without defects of congenital origin.

5. Bone marrow transplantation is indicated for many and especially for combined IDS (SCID, Wiskott-Aldrich syndrome).

6. Gene therapy - adenosine deaminase transplantation (performed on several patients in Europe and the USA).

2. Secondary immunodeficiency states

Secondary immunodeficiency states are characterized by a violation of humoral and cellular immunity, the synthesis of complement components, the absence or decrease in the activity of cytotoxic lymphocytes and macrophages. In childhood, they lead to the breakdown of post-vaccination immunity and the ineffectiveness of vaccination programs.

Secondary immunodeficiency states are disorders of the immune system that develop in the postneonatal period in children or adults and are not the result of genetic defects. Causes leading to the development of secondary immunodeficiency states: nutritional deficiencies, chronic viral and bacterial infections, chemo- and corticosteroid therapy, irrational use of drugs, age-related atrophy of the thymus, exposure to radiation, unbalanced diet, poor-quality drinking water, extensive surgical operations, excessive physical activity , multiple injuries, stress, exposure to pesticides, other environmental factors.

Classification. Classification of secondary immunodeficiency states.

1. Systemic, developing as a result of damage to immunogenesis (with radiation, toxic, infectious and stress lesions).

2. Local, characterized by regional damage to immunocompetent cells (local disorders of the immune apparatus of mucous membranes, skin and other tissues, developed as a result of local inflammatory, atrophic and hypoxic disorders).

Diseases accompanied by secondary immunodeficiency conditions.

1. Infectious diseases: protozoal and helminthic diseases; bacterial, viral and fungal infections.

2. Nutritional disorders: exhaustion, cachexia, malabsorption syndrome, etc.

3. Exogenous and endogenous intoxications - with renal and hepatic insufficiency, with poisoning, etc.

4. Tumors of lymphoreticular tissue (lymphocytic leukemia, thymoma, granulomatosis and other neoplasms).

5. Metabolic diseases (diabetes mellitus).

6. Protein loss due to intestinal diseases, nephrotic syndrome, burn disease, etc.

7. The action of various types of radiation.

8. Strong prolonged stress.

9. The action of drugs.

10. Blockade by immune complexes and antibodies of lymphocytes in allergic and autoimmune diseases.

Treatment.

Replacement therapy with various immune drugs (immunoglobulins; antitoxic, anti-influenza and anti-staphylococcal serums). Correction of the effector link with pharmacological drugs (decaris, diucefon, imuran, cyclophosphamide, etc.), hormones and mediators of the immune system (leukocyte interferons, thymus preparations - thymosin, thymalin, T-activin). Removal of inhibitory factors that bind antibodies and block the effect of immunocorrection (hemosorption, plasmapheresis, hemodialysis, lymphpheresis).

1. In violation of the T-cell link: T-activin, thymogen, interferon, interleukin-2, levamisole, cimetidine, azimixon, imutiol, isoprecasin, etc.

2. For a defect in the macrophage link: MDP (muramyl dipeptide), lentinan, zymosan, aubzidan, peptolak, lactolene, interferon-A, prodigiosan, etc.

3. In case of insufficiency or defects in B-cell immunity - activation of T-helpers and macrophages.

4. With a defect in the complement system - plasma transfusion

5. For immunodeficiencies resulting from radiotherapy and chemotherapy, agents for stimulating the bone marrow: granulocyte-macrophage colony-stimulating factor (GM-CSF), granulocyte colony-stimulating factor (G-CSF), interleukin-1 and interleukin-3.

Correction of transient forms of secondary immunodeficiency states.

1. Achievement of the period of remission:

1) antigenic loads are excluded (dissociation of the patient from the team);

2) sanitation of foci of infections in the family;

3) reducing the influence of other social factors;

4) it is necessary to exclude contact with allergens and vaccines;

5) therapeutic measures are organized on an outpatient basis;

6) detoxification and enteral sorption are carried out;

7) complexes of vitamins, microelements and various antioxidants are used;

8) individual anti-inflammatory measures (if necessary - antibacterial drugs);

9) restoration of the functions of the gastrointestinal tract.

2. When the activity of pathological processes subsides:

1) immunotropic drugs containing factors of the thymus and bone marrow (taktavin, thymalin, thymogen, myelopid) are prescribed;

2) the choice of a specific drug is based on tests for determining the sensitivity of lymphocytes to drugs;

3) other drugs of this plan are prescribed only after the previous stage of rehabilitation of patients;

4) with an individual choice of a drug, the achievement of a positive result of therapy becomes natural;

5) it becomes possible to prevent acute and exacerbations of chronic diseases.

3. Treatment of the underlying pathology:

1) in case of neurological pathology - drugs that improve microcirculation and metabolic processes in the central nervous system, antihypertensive and diuretic drugs;

2) with primary vegetovascular dystonia - psychotherapy of family members; decrease in the predominant activity of the departments of the ANS;

3) for metabolic and constitutional disorders - membrane stabilizing agents, diet;

4) in chronic infectious processes - antibacterial, antiviral, antifungal and antiparasitic drugs; inducers of nonspecific defense of the body;

5) for all patients - drugs that improve metabolic processes.

3. Principles of therapy for immunopathological syndromes

With the syndrome of violation of anti-infectious protection - adequate antibacterial, antiviral, antifungal and antiparasitic therapy; immunomodulatory drugs; rational mode of antigenic loads, including vaccination.

Treatment of allergy syndrome - separation from causally significant allergens, elimination of metabolic products, enzyme replacement therapy, hyposensitizing effects, immunomodulatory measures according to an individual program. In the treatment of diseases associated with the development of autoimmune syndrome, antibacterial therapy is futile; cytostatics, extracorporeal treatment methods, and intravenous administration of immunoglobulin-containing drugs are indicated.

In the stage of subsidence of the inflammatory process - immunomodulatory therapy and drugs containing cytokines - leukinferon, interferon, reaferon, etc. In all cases - sanitation of foci of chronic infections.

Author: Pavlova N.V.

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