Hypogammaglobulinemia

Hypogammaglobulinemia
Specialty Hematology Edit this on Wikidata

Hypogammaglobulinemia is a type of primary immunodeficiency disease[1] in which not enough gamma globulins exist in the blood (thus hypo- + gamma + globulin + -emia). This entails that not enough antibodies exist, which impairs the immune system. Hypogammaglobulinemia is a characteristic of common variable immunodeficiency.[2] Other causes include loss of gamma globulins in urine, as in nonselective glomerular proteinuria.

Diagnosis

Types

Type OMIM Gene
AGM1 601495 IGHM
AGM2 613500 IGLL1
AGM3 613501 CD79A
AGM4 613502 BLNK
AGM5 613506 LRRC8A
AGM6 612692 CD79B

Treatment

Treatment is by parenteral administration of gamma globulins, either monthly intravenously,subcutaneously, or more recently, by weekly self-administered hypodermoclysis. In either case, mild allergic reactions (generalized pruritus, urticaria) are common, and are usually manageable with oral diphenhydramine.

Etymology

"Hypogammaglobulinemia" is largely synonymous with "agammaglobulinemia". When the latter term is used (as in "X-linked agammaglobulinemia") 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,[3] but the distinction is not usually clinically relevant.

"Hypogammaglobulinemia" is distinguished from dysgammaglobulinemia, which is a reduction in some types of gamma globulins, but not others.[4]

References

  1. ↑ "hypogammaglobulinemia" at Dorland's Medical Dictionary
  2. ↑ "common variable immunodeficiency" at Dorland's Medical Dictionary
  3. ↑ "agammaglobulinemia" at Dorland's Medical Dictionary
  4. ↑ "Dysgammaglobulinemia" at Dorland's Medical Dictionary

Further reading

  • Rose, M. E.; Lang, D. M. (2006). "Evaluating and managing hypogammaglobulinemia". Cleveland Clinic journal of medicine. 73 (2): 133–7, 140, 143–4. PMID 16478038.
  • Robert Y Li, et al.: "Hypogammaglobulinemia", Medscape. Accessed 2009-07-17.
Classification
External resources


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