Hypergammaglobulinemia: Difference between revisions

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VisualWikitext

Latest revision as of 21:08, 2 March 2016

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	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

↑ ^1.0 ^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

Related Chapters

Monoclonal gammopathy of undetermined significance

Template:WikiDoc Sources

[kb-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

[OMIM-6] Immunodeficiency with hyper-IgM, Type 1; HIGM1. http://www.omim.org/entry/308230. Accessed on February 24, 2016

[1]

[2]

[3]

[4]

[5]

[6]

@@ Line 1: / Line 1: @@
 __NOTOC__
+{{SI}}
 {{CMG}}{{AE}} {{STM}}
-{{SK}}
+{{SK}} Hyper IgM syndrome; Hyper IgD syndrome; Hyper IgE syndrome; HIES; Job syndrome; Buckley syndrome
 ==[[Hypergammaglobulinemia 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><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>
 ==[[Hypergammaglobulinemia classification|Classification]]==
+*Hypergammaglobulinemia may be classified into the following:
+**[[Hyper IgM syndrome]]s
+**Hyper IgD syndrome
+**[[Hyper IgE 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==
@@ Line 14: / Line 64: @@
 *[[Monoclonal gammopathy of undetermined significance]]
 {{Immune disorders}}
 [[Category:Rheumatology]]
 [[Category:Immunology]]
-[[Category:Needs content]]
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See also hematological malignancy and hematology