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IMMUNODEFICIENCY

(Redirected from Immune-system deficiencies)

In medicine, 'immunodeficiency' (or 'immune deficiency') is a state in which the immune system's ability to fight infectious disease is compromised or entirely absent. Most cases of immunodeficiency are either congenital or acquired. A person who has an immunodeficiency is said to be 'immunocompromised'. An immunocompromised person is very vulnerable to opportunistic infections. Boys more often than girls can have immune deficiency. Mostly immune deficiency is recognized in the early ages unless it is an acquired immune deficiency.

Contents
Congenital (or Primary) immune deficiency
Acquired immune deficiency
Biochemistry

Congenital (or Primary) immune deficiency


A number of rare diseases feature a heightened susceptibility to infections from childhood onward. Many of these disorders are hereditary and autosomal recessive.
The following is a list of the congenital immunodeficiencies organized by the immune component that is impaired or missing.

B cell deficiencies


X-linked agammaglobulinemia


Selective Immunoglobulin Deficiency

T cell deficiencies


DiGeorge's syndrome (Thymic aplasia)


★ Chronic mucocutaneous candidiasis


Hyper-IgM syndrome


Interleukin-12 receptor deficiency

★ Combined T cell & B cell abnormalities


Severe Combined Immunodeficiency Disease (SCID)


Wiskott-Aldrich syndrome


Ataxia-telangiectasia

Complement Deficiencies


Hereditary Angioedema or Hereditary angioneurotic edema


★ Recurrent Infections


★ Autoimmune-like diseases


Paroxysmal nocturnal hemoglobinuria

Phagocyte deficiencies


Leukocyte adhesion deficiency


Chronic Granulomatous Disease (CGD)


Chédiak-Higashi syndrome


Job's syndrome (Hyper-IgE syndrome)


Cyclic neutropenia


Myeloperoxidase deficiency


Glucose-6-phosphate dehydrogenase deficiency


Interferon-γ deficiency

★ Various / Unknown


Common Variable Immunodeficiency (CVID)


Vici syndrome

Acquired immune deficiency


Although elderly people are generally more susceptible to infections, this can be increased in the presence of a number of blood disorders (see hematology); important examples are multiple myeloma and chronic lymphatic leukemia (CLL).
Immune suppression is a common side-effect of many drugs, most importantly chemotherapy. However, Disease-modifying antirheumatic drugs (DMARDs) are used to suppress immune activity in autoimmune disorders to slow progression of this type of disease. Any drug that causes neutropenia or agranulocytosis can cause immune suppression and sepsis. Organ transplant recipients are given immunosuppressive drugs to avoid transplant rejection.
Immune deficiency is also the hallmark of Acquired Immunodeficiency Syndrome (AIDS), caused by a virus, Human immunodeficiency virus (HIV). HIV destroys CD4+-T-lymphocytes (Helper T cells), which play a critical role in modulating immune response, and the loss of these immune cells leads eventually to immunocompromise and opportunistic infections.

Biochemistry


In all higher mammals a complex set of biochemicals is present that regulates the immune system. Imbalances of these chemicals by nutritional deficit, disease or infection can compromise the immune system.

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