Hypergammaglobulinemia
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]
Synonyms and keywords: Hyper IgM syndrome; Hyper IgD syndrome; Hyper IgE syndrome; HIES; Job syndrome; Buckley syndrome
Overview
Hypergammaglobulinemia is a primary immunodeficiency disease with an elevated concentration of gamma globulins (immunoglobulins or antibodies).[1][2][3] The term "hypergammaglobulinemia" refers to the position of the excess of proteins after serum protein electrophoresis (found in the gammaglobulin region).[1] The majority of hypergammaglobulinemias are due to an excess of immunoglobulin M (IgM) because this is the default immunoglobulin type prior to class switching.[1] Hypergammaglobulinemias may also result from a deficiency in the other major types of immunoglobulins (IgA, IgE, and IgG).[1][4]
Classification
- Hypergammaglobulinemia may be classified into the following:
- Hyper IgM syndromes
- Hyper IgD syndrome
- Hyper IgE syndrome
- Hyper IgM associated hypergammaglobulinemias are further classified into the following five types:[5][6]
| Type | Gene involved | Mode of inheritance |
|---|---|---|
|
Type 1 |
|
X-linked recessive |
|
Type 2 |
|
Autosomal recessive |
|
Type 3 |
|
Autosomal recessive |
|
Type 4 |
|
|
|
Type 5 |
|
Autosomal recessive |
References
- ↑ 1.0 1.1 1.2 1.3 Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.
- ↑ Globulin. Wikipedia. https://en.wikipedia.org/wiki/Globulin. Wikipedia. Accessed on February 24, 2016.
- ↑ Primary immunodeficiency. Wikipedia. https://en.wikipedia.org/wiki/Primary_immunodeficiency Accessed on March 2, 2016.
- ↑ Immunoproliferative disorder. Wikipedia. Accessed on February 24, 2016.
- ↑ Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016
- ↑ Immunodeficiency with hyper-IgM, Type 1; HIGM1. http://www.omim.org/entry/308230. Accessed on February 24, 2016