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==Overview==
==Overview==
Hypergammaglobulinemia is a [[primary immunodeficiency]] disease with an elevated concentration of [[gamma globulin]]s ([[immunoglobulin]]s or [[antibodies]]).<ref name=kb>Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.</ref><ref>Globulin. Wikipedia. https://en.wikipedia.org/wiki/Globulin. Wikipedia. Accessed on February 24, 2016.</ref> The term "hypergammaglobulinemia" refers to the position of the excess of proteins after [[serum protein electrophoresis]] (found in the gammaglobulin region).<ref name=kb>Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.</ref> The majority of hypergammaglobulinemias are caused by an excess of [[immunoglobulin]] M (IgM) because this is the default [[immunoglobulin]] type prior to [[class switching]].<ref name=kb>Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.</ref> Hypergammaglobulinemias may also be caused by a deficiency in the other major types of [[immunoglobulin]]s.<ref name=kb>Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.</ref><ref>Immunoproliferative disorder. Wikipedia. Accessed on February 24, 2016.</ref>
Hypergammaglobulinemia is a [[primary immunodeficiency]] disease with an elevated concentration of [[gamma globulin]]s ([[immunoglobulin]]s or [[antibodies]]).<ref name=kb>Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.</ref><ref>Globulin. Wikipedia. https://en.wikipedia.org/wiki/Globulin. Wikipedia. Accessed on February 24, 2016.</ref><ref>Primary immunodeficiency. Wikipedia. https://en.wikipedia.org/wiki/Primary_immunodeficiency Accessed on March 2, 2016.</ref> The term "hypergammaglobulinemia" refers to the position of the excess of proteins after [[serum protein electrophoresis]] (found in the [[gammaglobulin]] region).<ref name=kb>Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.</ref> The majority of hypergammaglobulinemias are due to an excess of [[immunoglobulin]] M ([[IgM]]) because this is the default [[immunoglobulin]] type prior to [[class switching]].<ref name=kb>Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.</ref> Hypergammaglobulinemias may also result from a deficiency in the other major types of [[immunoglobulin]]s ([[IgA]], [[IgE]], and [[IgG]]).<ref name=kb>Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.</ref><ref>Immunoproliferative disorder. Wikipedia. Accessed on February 24, 2016.</ref>


==Classification==
==Classification==
*Hypergammaglobulinemia may be classified into the following:
**Hyper Ig M associated hypergammaglobulinemias
**Hyper Ig D syndrome
**Hyper Ig E syndrome
*Hyper IgM associated hypergammaglobulinemias are further classified into the following five types:<ref>Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016</ref><ref name=OMIM>Immunodeficiency with hyper-IgM, Type 1; HIGM1. http://www.omim.org/entry/308230. Accessed on February 24, 2016</ref>
{| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
|valign=top|
|+
! style="background: #4479BA; width: 150px;" | {{fontcolor|#FFF|Type}}
! style="background: #4479BA; width: 370px;" | {{fontcolor|#FFF|Gene involved}}
! style="background: #4479BA; width: 150px;" | {{fontcolor|#FFF|Mode of inheritance}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center |
Type 1
| style="padding: 5px 5px; background: #F5F5F5;" |
*Tumor Necrosis Factor Super Family member 5 (''TNFSF5'') gene encoding CD40L
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center |
X-linked recessive
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
Type 2
| style="padding: 5px 5px; background: #F5F5F5;" |
*Activation-Induced Cytidine Deaminase (''AICDA'') gene
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center |
Autosomal recessive
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
Type 3
| style="padding: 5px 5px; background: #F5F5F5;" |
*''CD40'' gene
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center |
Autosomal recessive
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
Type 4
| style="padding: 5px 5px; background: #F5F5F5;" |
*Not yet known
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center |
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
Type 5
| style="padding: 5px 5px; background: #F5F5F5;" |
*Gene encoding Uracil-DNA glycosylase
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center |
Autosomal recessive
|}
==References==
==References==
{{reflist|2}}
{{reflist|2}}

Latest revision as of 18:57, 2 March 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]

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 Ig M associated hypergammaglobulinemias
    • Hyper Ig D syndrome
    • Hyper Ig E syndrome
  • Hyper IgM associated hypergammaglobulinemias are further classified into the following five types:[5][6]
Type Gene involved Mode of inheritance

Type 1

  • Tumor Necrosis Factor Super Family member 5 (TNFSF5) gene encoding CD40L

X-linked recessive

Type 2

  • Activation-Induced Cytidine Deaminase (AICDA) gene

Autosomal recessive

Type 3

  • CD40 gene

Autosomal recessive

Type 4

  • Not yet known

Type 5

  • Gene encoding Uracil-DNA glycosylase

Autosomal recessive

References

  1. ↑ 1.0 1.1 1.2 1.3 Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016.
  2. ↑ Globulin. Wikipedia. https://en.wikipedia.org/wiki/Globulin. Wikipedia. Accessed on February 24, 2016.
  3. ↑ Primary immunodeficiency. Wikipedia. https://en.wikipedia.org/wiki/Primary_immunodeficiency Accessed on March 2, 2016.
  4. ↑ Immunoproliferative disorder. Wikipedia. Accessed on February 24, 2016.
  5. ↑ Hypergammaglobulinemia. Wikipedia. https://en.wikipedia.org/wiki/Hypergammaglobulinemia Accessed on February 24, 2016
  6. ↑ Immunodeficiency with hyper-IgM, Type 1; HIGM1. http://www.omim.org/entry/308230. Accessed on February 24, 2016

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