Dysgammaglobulinemia

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Dysgammaglobulinemia
immunoglobulin (antibody)
Pronunciation
  • dis-gam″ә-glob″u-lin-e´me-ә[1]
SpecialtyHematology Edit this on Wikidata
TypesSelective immunoglobulin A deficiency, Selective IgE deficiency disease, IgG deficiency, and Isolated primary immunoglobulin M deficiency.

Dysgammaglobulinemia is a type of immune disorder characterized by a reduction in some types of gamma globulins, resulting in heightened susceptibility to some infectious diseases where primary immunity is antibody based.[1]

It is distinguished from hypogammaglobulinemia, which is a reduction in all types of gamma globulins.[2]

There are four main types of dysgammaglobulinemia.[3]

Types

  • Selective immunoglobulin A deficiency - 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.[4]
  • 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.[5]
  • 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.[6] Those with IgG deficiency often get chronic or reoccurring pyogenic respiratory tract infections.[7]
  • 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.[8]

See also

References

  1. ^ a b "Dysgammaglobulinemia" at Dorland's Medical Dictionary
  2. ^ "hypogammaglobulinemia" at Dorland's Medical Dictionary
  3. ^ "Monarch Initiative". monarchinitiative.org. Retrieved 2023-11-27.
  4. PMID 20101521
    .
  5. . Retrieved 2023-11-27.
  6. .
  7. ^ Schwartz, Robert A (June 30, 2023). "Immunoglobulin G Deficiency Clinical Presentation: History, Physical, Causes". Medscape Reference. Retrieved November 27, 2023.
  8. PMID 28928736
    .

Further reading

External links