Dysgammaglobulinemia Explained

Dysgammaglobulinemia
Pronounce:	dis-gam″ә-glob″u-lin-e´me-ә
Types:	Selective 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.

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

There are four main types of dysgammaglobulinemia.^[1]

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.^[2]
• 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.^[3]
• 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.^[4] Those with IgG deficiency often get chronic or reoccurring pyogenic respiratory tract infections.^[5]
• 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.^[6]

See also

• Immunodeficiency
• Hypogammaglobulinemia

Further reading

• Book: Cheraghi . Taher . Kalantari . Arash . Shabestari . Mahnaz Sadeghi . Abolhassani . Hassan . Eibel . Hermann . Hammarström . Lennart . Kanegane . Hirokazu . Durandy . Anne . Plebani . Alessandro . Cunningham-Rundles . Charlotte . Aghamohammadi . Asghar . Inborn Errors of Immunity . Predominantly antibody deficiencies . Elsevier . 2021 . 978-0-12-821028-4 . 10.1016/b978-0-12-821028-4.00006-3. none. 2023-11-27.
• Book: van der Burg . Mirjam . Dalm . Virgil A.S.H. . Weemaes . Corry M.R. . Stiehm's Immune Deficiencies . Isotype defects . Elsevier . 2020 . 978-0-12-816768-7 . 10.1016/b978-0-12-816768-7.00020-x. 241231704 . none. 2023-11-27.

External links

• Dysgammaglobulinemia: Monarch Initiative Disease Ontology Dorland's Medical Dictionary
• Selective immunoglobulin A deficiency: Cleveland Clinic MerckManual EMedicine DermNet Mayo Clinic
• Selective IgE deficiency disease: Monarch Initiative Disease Ontology NCI Thesaurus
• IgG deficiency: Cedars Sinai Johns Hopkins EMedicine UpToDate
• Isolated primary immunoglobulin M deficiency: GARD Immune Deficiency Foundation UpToDate EMedicine

Notes and References

1. Web site: 2023-11-27. Monarch Initiative. monarchinitiative.org.
2. Yel . Leman . Selective IgA Deficiency . Journal of Clinical Immunology . Springer Science and Business Media LLC . 30 . 1 . 2010 . 0271-9142 . 10.1007/s10875-009-9357-x . 10–16. free. 20101521 . 2821513 .
3. Magen . Eli . Schlesinger . Menachem . David . Michael . Ben-Zion . Itzhak . Vardy . Daniel . Selective IgE deficiency, immune dysregulation, and autoimmunity . Allergy and Asthma Proceedings . Oceanside Publications Inc. . 35 . 2 . March 1, 2014 . 1088-5412 . 10.2500/aap.2014.35.3734 . 27–33. 24717782 . 2023-11-27.
4. Agarwal . Shradha . Cunningham-Rundles . Charlotte . Assessment and clinical interpretation of reduced IgG values . Annals of Allergy, Asthma & Immunology . Elsevier BV . 99 . 3 . 2007 . 1081-1206 . 10.1016/s1081-1206(10)60665-5 . 281–283. free. 17910333 . 3099256 .
5. Web site: Schwartz . Robert A . Immunoglobulin G Deficiency Clinical Presentation: History, Physical, Causes . Medscape Reference . June 30, 2023 . November 27, 2023.
6. Gupta . Sudhir . Gupta . Ankmalika . Selective IgM Deficiency—An Underestimated Primary Immunodeficiency . Frontiers in Immunology . Frontiers Media SA . 8 . September 5, 2017 . 1056 . 1664-3224 . 10.3389/fimmu.2017.01056 . free. 28928736 . 5591887 .