Hypogammaglobulinemia

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]

Terminology

"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]

Types

Treatment

Treatment is by parenteral administration of gamma globulins, either monthly intravenously, 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.

References

External links

• "Hypogammaglobulinemia" at Dorland's Medical Dictionary

• 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.
• Saul Greenberg: "Hypogammaglobulinemia ". Accessed 2009-07-17.