Hypergammaglobulinemia overview: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
No edit summary
 
(One intermediate revision by the same user not shown)
Line 4: Line 4:


==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 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 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

Hypergammaglobulinemia Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hypergammaglobulinemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Hypergammaglobulinemia overview On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Hypergammaglobulinemia overview

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Hypergammaglobulinemia overview

CDC on Hypergammaglobulinemia overview

Hypergammaglobulinemia overview in the news

Blogs on Hypergammaglobulinemia overview

Directions to Hospitals Treating Hypergammaglobulinemia

Risk calculators and risk factors for Hypergammaglobulinemia overview

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

Related Chapters

Template:WikiDoc Sources

Retrieved from "https://www.wikidoc.org/index.php?title=Hypergammaglobulinemia_overview&oldid=1221326"