Hypogammaglobulinemia
| Hypogammaglobulinemia | |
|---|---|
| Classification and external resources | |
| Specialty | hematology |
| ICD-10 | D80.0-D80.1 |
| ICD-9-CM | 279.00 |
| DiseasesDB | 6426 |
| MedlinePlus | 001307 |
| eMedicine | med/1120 ped/54 |
| Patient UK | Hypogammaglobulinemia |
| MeSH | D000361 |
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
| 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, 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
- 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.