Dysgammaglobulinemia is a type of
immune disorder
An immune disorder is a dysfunction of the immune system. These disorders can be characterized in several different ways:
* By the component(s) of the immune system affected
* By whether the immune system is overactive or underactive
* By whether ...
characterized by a reduction in some types of
gamma globulin
Gamma globulins are a class of globulins, identified by their position after serum protein electrophoresis. The most significant gamma globulins are immunoglobulins (antibodies), although some immunoglobulins are not gamma globulins, and some ...
s, resulting in heightened susceptibility to some infectious diseases where primary immunity is antibody based.
It is distinguished from
hypogammaglobulinemia
Hypogammaglobulinemia is an immune system disorder in which not enough gamma globulins are produced in the blood (thus '' hypo-'' + ''gamma'' + '' globulin'' + '' -emia''). This results in a lower antibody count, which impairs the immune system, ...
, which is a reduction in ''all'' types of gamma globulins.
There are four main types of dysgammaglobulinemia.
Types
*
Selective immunoglobulin A deficiency
Selective immunoglobulin A (IgA) deficiency (SIgAD) is a kind of immunodeficiency, a type of hypogammaglobulinemia. People with this deficiency lack immunoglobulin A (IgA), a type of antibody that protects against infections of the mucous membran ...
- Selective immunoglobulin A deficiency is classified as an IgA level below 7mg/dl (0.4375 μmol/L) with normal levels of other immunoglobulins. Some people with selective immunoglobulin A deficiency are prone to infections and develop other autoimmune disorders such as lupus, celiac disease and inflammatory bowel disease while others are completely asymptomatic.
*
Selective IgE deficiency disease - Selective IgE deficiency disease is characterized by IgE levels below <2 kIU/L with normal levels of other immunoglobulins. Selective IgE deficiency disease may be associated with an increased predisposition to certain sinopulmonary diseases, autoimmune disorders, infections, and malignancies.
*
IgG deficiency - IgG deficiency is a lack of IgG in the absence of other immunoglobulin deficiencies. IgG levels of 300–600 mg/dL show a mild to moderate reduction of IgG while levels below 200 mg/dl are classified as a severe deficiency.
Those with IgG deficiency often get chronic or reoccurring pyogenic respiratory tract infections.
*
Isolated primary immunoglobulin M deficiency - Isolated primary immunoglobulin M deficiency is classified as IgM levels under two SD of mean with normal levels of other immunoglobulins. Isolated primary immunoglobulin M deficiency often presents as recurring infections. Patients often improve with immunoglobulin therapy.
See also
*
Immunodeficiency
Immunodeficiency, also known as immunocompromise, is a state in which the immune system's ability to fight infectious diseases and cancer is compromised or entirely absent. Most cases are acquired ("secondary") due to extrinsic factors that aff ...
*
Hypogammaglobulinemia
Hypogammaglobulinemia is an immune system disorder in which not enough gamma globulins are produced in the blood (thus '' hypo-'' + ''gamma'' + '' globulin'' + '' -emia''). This results in a lower antibody count, which impairs the immune system, ...
References
Further reading
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External links
* Dysgammaglobulinemia
Monarch InitiativeDisease Ontology
* Selective immunoglobulin A deficiency
Cleveland ClinicMerckManualEMedicineDermNetMayo Clinic
* Selective IgE deficiency disease
Monarch InitiativeDisease OntologyNCI Thesaurus
* IgG deficiency
Johns HopkinsEMedicineUpToDate
* Isolated primary immunoglobulin M deficiency
GARDImmune Deficiency FoundationUpToDateEMedicine
{{Set index article
Predominantly antibody deficiencies