Primary immunodeficiency states belong to the group of severe genetically determined diseases caused by the violation of one or more immune defense mechanisms. Most of these conditions make their debut in early childhood with an increased susceptibility to infectious diseases.

Secondary immunodeficiencies are the result of serious external effects on the body: radiation, immunosuppressive chemotherapy, severe infections (for example, HIV). Compared with the first group, they are much more common, however, as a rule, they don’t cause diagnostic problems, as they are the result of already known factors.

The clinic has a number of common features:

  1. Recurrent and chronic infections of the upper respiratory tract, sinuses, skin, mucous membranes, gastrointestinal tract, often caused by opportunistic bacteria, protozoa, fungi that have a tendency to generalization, septicemia and torpid to conventional therapy.
  2. Hematological deficiencies: leukocytopenia, thrombocytopenia, anemia (hemolytic and megaloblastic).
  3. Autoimmune disorders: SLE (Systemic lupus erythematosus) -like syndrome, arthritis, systemic scleroderma, chronic active hepatitis, thyroiditis.
  4. Often immunodeficiency is combined with allergic reactions of type 1 in the form of eczema, angioedema, allergic reactions to the introduction of drugs, immunoglobulin, and blood.
  5. Tumors and lymphoproliferative diseases with immunodeficiency occur 1,000 times more often than without immunodeficiency.
  6. Digestive disorders, diarrhea syndrome and malabsorption syndrome are often noted in patients with immunodeficiency.
  7. Patients with immunodeficiency are characterized by unusual reactions to vaccination, and the use of live vaccines in them is dangerous for the development of sepsis.
  8. Primary immunodeficiency are often combined with developmental defects, primarily with hypoplasia of the cellular elements of cartilage and hair. Cardiovascular defects are described mainly in the D-Georges syndrome.
Affected components Main causes
Humoral immune deficiency
B cell deficiency
Bcells, plasma cellsor antibodies
  • Primary humoral
  • Multiple myeloma
  • Chronic lymphoid leukemia
  • AIDS
T cell deficiency T cells
  • Marrow and other transplantation
  • AIDS
  • Cancer chemotherapy
  • Lymphoma
  • Glucocorticoid therapy
Neutropenia Neutrophil granulocytes
  • Chemotherapy
  • Bone marrow transplantation
  • Dysfunction, such as chronic granulomatous disease
Asplenia Spleen
  • Splenectomy
  • Trauma
  • Sickle-cell anemia
Complement deficiency Complement system
  • Congenital deficiencies

Primary Immunodeficiency Disease Symptoms & Diagnosis:

  • Recurrent, unusual or difficult to treat infections
  • Poor growth or loss of weight
  • Recurrent pneumonia, ear infections or sinusitis
  • Multiple courses of antibiotics or IV antibiotics necessary to clear infections
  • Recurrent deep abscesses of the organs or skin
  • A family history of Immunodeficiency Disease
  • Swollen lymph glands or an enlarged spleen
  • Autoimmune disease

As a rule, secondary immunodeficiencies are transient. In this regard, the treatment of secondary immunodeficiencies is much simpler and more effective compared with the treatment of primary disorders of the immune system function. Usually, treatment of secondary immunodeficiency begins with identifying and eliminating the cause of its occurrence. For example, treatment of immunodeficiency against the background of chronic infections begins with the rehabilitation of foci of chronic inflammation. Immunodeficiency on the background of vitamin and mineral deficiencies begin to be treated with the help of vitamins and minerals. The regenerative ability of the immune system is great, so eliminating the causes of immunodeficiency usually leads to the restoration of the immune system. To speed up recovery and stimulate the immune system, a course of treatment including immunomodulatory STF is carried out.

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