Dysgammaglobulinemia

SpecialtyHematology 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

• Immunodeficiency
• Hypogammaglobulinemia

References

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