Hypogammaglobulinemia
Encyclopedia
Hypogammaglobulinemia is a type of immune disorder
Immune disorder
An immune disorder is a dysfunction of the immune system. These disorders can be characterized in several different ways:* By the component of the immune system affected* By whether the immune system is overactive or underactive...

 characterized by a reduction in all types of gamma globulin
Gamma globulin
Gamma globulins are a class of globulins, identified by their position after serum protein electrophoresis. The most significant gamma globulins are immunoglobulins , more commonly known as antibodies, although some Igs are not gamma globulins, and some gamma globulins are not Igs.-Use as medical...

s.

Hypogammaglobulinemia is a characteristic of common variable immunodeficiency
Common variable immunodeficiency
Common variable immunodeficiency is a group of approximately 150 primary immunodeficiencies , which have a common set of symptoms but which have different underlying causes.Common variable immunodeficiency is the most commonly encountered primary immunodeficiency.-Causes and types:CVID...

.

Terminology

"Hypogammaglobulinemia" is distinguished from dysgammaglobulinemia
Dysgammaglobulinemia
Dysgammaglobulinemia is a type of immune disorder characterized by a reduction in some types of gamma globulins.It is distinguished from hypogammaglobulinemia, which is a reduction in all types of gamma globulins....

, which is a reduction in some types of gamma globulin
Gamma globulin
Gamma globulins are a class of globulins, identified by their position after serum protein electrophoresis. The most significant gamma globulins are immunoglobulins , more commonly known as antibodies, although some Igs are not gamma globulins, and some gamma globulins are not Igs.-Use as medical...

s, but not others.

"Hypogammaglobulinemia" is largely synonymous with "agammaglobulinemia". When this term is used (as in "X-linked agammaglobulinemia
X-linked agammaglobulinemia
X-linked agammaglobulinemia is a rare X-linked genetic disorder that was discovered in 1952 which affects the body's ability to fight infection. XLA is an X-linked disorder, and therefore is more common in males...

") it implies that gamma globulins are not merely reduced, but completely absent. Modern assays have allowed most agammaglobulinemias to be more precisely defined as hypogammaglobulinemias, but the distinction is not usually clinically relevant.

Types

Type OMIM Gene
AGM1 IGHM
IGHM
Ig mu chain C region is a protein that in humans is encoded by the IGHM gene.It is associated with agammaglobulinemia-1.-Further reading:...

AGM2 IGLL1
IGLL1
Immunoglobulin lambda-like polypeptide 1 is a protein that in humans is encoded by the IGLL1 gene. IGLL1 has also recently been designated CD179B .It is associated with agammaglobulinemia-2.-Further reading:...

AGM3 CD79A
CD79A
CD79a molecule, immunoglobulin-associated alpha, also known as mb-1, is a human gene.The mb-1 gene codes for a phosphoprotein, designated CD79a CD79a molecule, immunoglobulin-associated alpha, also known as mb-1, is a human gene.The mb-1 gene codes for a phosphoprotein, designated CD79a CD79a...

AGM4 BLNK
AGM5 LRRC8A
LRRC8A
Leucine-rich repeat-containing protein 8A is a protein that in humans is encoded by the LRRC8A gene.It is associated with agammaglobulinemia-5.-Further reading:...

AGM6 CD79B
CD79B
CD79b molecule, immunoglobulin-associated beta, also known as CD79B , is a human gene.It is associated with agammaglobulinemia-6.-External links:...


External links

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