- Hypogammaglobulinemia
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Hypogammaglobulinemia Classification and external resources ICD-10 D80.0-D80.1 ICD-9 279.00 DiseasesDB 6426 MedlinePlus 001307 eMedicine med/1120 ped/54 MeSH D000361 Hypogammaglobulinemia is a type of immune disorder characterized by a reduction in all types of gamma globulins.[1]
Hypogammaglobulinemia is a characteristic of common variable immunodeficiency.[2]
Contents
Terminology
"Hypogammaglobulinemia" is distinguished from dysgammaglobulinemia, which is a reduction in some types of gamma globulins, but not others.[3][4]
"Hypogammaglobulinemia" is largely synonymous with "agammaglobulinemia". When this 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,[5] but the distinction is not usually clinically relevant.
Types
Type OMIM Gene AGM1 601495 IGHM AGM2 613500 IGLL1 AGM3 613501 CD79A AGM4 613502 BLNK AGM5 613506 LRRC8A AGM6 612692 CD79B References
- ^ "hypogammaglobulinemia" at Dorland's Medical Dictionary
- ^ "common variable immunodeficiency" at Dorland's Medical Dictionary
- ^ "Dysgammaglobulinemia" at Dorland's Medical Dictionary
- ^ Dysgammaglobulinemia at eMedicine Dictionary
- ^ "agammaglobulinemia" at Dorland's Medical Dictionary
External links
- "Hypogammaglobulinemia" at Dorland's Medical Dictionary
- Mark E. Rose & David M. Lang: "Evaluating and managing hypogammaglobulinemia", Cleveland Clinic Journal of Medicine, Vol. 73, No. 2 (February 2006), pp. 133–144. Accessed 2009-07-17.
- Robert Y Li, et al.: "Hypogammaglobulinemia", Medscape. Accessed 2009-07-17.
- Saul Greenberg: "Hypogammaglobulinemia ". Accessed 2009-07-17.
Immune disorders: Lymphoid and complement immunodeficiency (D80–D85, 279.0–4) Primary HypogammaglobulinemiaIgA deficiency · IgG deficiency · IgM deficiency · Hyper IgM syndrome (2, 3, 4, 5) · Wiskott-Aldrich syndrome · Hyper-IgE syndromeOtherT cell deficiency (T)thymic hypoplasia: hypoparathyroid (Di George's syndrome) · euparathyroid (Nezelof syndrome, Ataxia telangiectasia)x-linked: X-SCID
autosomal: Adenosine deaminase deficiency · Omenn syndrome · ZAP70 deficiency · Bare lymphocyte syndromeAcquired Leukopenia:
LymphocytopeniaComplement deficiency C1-inhibitor (Angioedema/Hereditary angioedema) · Complement 2 deficiency/Complement 4 deficiency · MBL deficiency · Properdin deficiency · Complement 3 deficiency · Terminal complement pathway deficiency · Paroxysmal nocturnal hemoglobinuria · Complement receptor deficiencyGlobin Globulin Serpin Serpinopathy: Alpha 1-antitrypsin deficiency · Antithrombin III deficiency · Hereditary angioedema · FENIBCategories:- Lymphocytic immune system disorders
- Medicine stubs
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