Dysgammaglobulinemia

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.

Dysgammaglobulinemia
Schematic diagram of the basic unit of immunoglobulin (antibody)
Pronunciation	dis-gam″ә-glob″u-lin-e´me-ә
Specialty	Hematology
Types	Selective immunoglobulin A deficiency, Selective IgE deficiency disease, IgG deficiency, and Isolated primary immunoglobulin M deficiency.

It is distinguished from hypogammaglobulinemia, 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 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
• Hypogammaglobulinemia

Further reading

• Cheraghi, Taher; Kalantari, Arash; Shabestari, Mahnaz Sadeghi; Abolhassani, Hassan; Eibel, Hermann; Hammarström, Lennart; Kanegane, Hirokazu; Durandy, Anne; Plebani, Alessandro; Cunningham-Rundles, Charlotte; Aghamohammadi, Asghar (2021). "Predominantly antibody deficiencies". Inborn Errors of Immunity. Elsevier. doi:10.1016/b978-0-12-821028-4.00006-3. ISBN 978-0-12-821028-4. Retrieved 2023-11-27.
• van der Burg, Mirjam; Dalm, Virgil A.S.H.; Weemaes, Corry M.R. (2020). "Isotype defects". Stiehm's Immune Deficiencies. Elsevier. doi:10.1016/b978-0-12-816768-7.00020-x. ISBN 978-0-12-816768-7. S2CID 241231704. Retrieved 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