Humoral immune deficiency
| Humoral immune deficiency | |
|---|---|
| File:Original antigenic sin.svg | |
| Classification and external resources | |
| Specialty | Lua error in Module:Wikidata at line 446: attempt to index field 'wikibase' (a nil value). |
| ICD-10 | D80 |
| ICD-9-CM | 279.0 |
| Patient UK | Humoral immune deficiency |
Humoral immune deficiencies are conditions which cause impairment of humoral immunity, which can lead to immunodeficiency. It can be mediated by insufficient number or function of B cells, the plasma cells they differentiate into (with these two potentially being caused by B cell lymphocytopenia), or the antibody secreted by the plasma cells.
They are associated with increased vulnerability to infection, but can be difficult to detect (or asymptomatic) in the absence of infection.
They can be associated with increased risk of gastric cancer.[1]
Causes
One way of classifying humoral immune deficiencies are into primary (or hereditary) versus secondary (or acquired) ones.
Primary
The International Union of Immunological Societies classifies primary immune deficiencies of the humoral system (here termed "Predominantly antibody deficiencies") as follows:[2]
- Absent B cells with a resultant severe reduction of all types of antibody: X-linked agammaglobulinemia (btk deficiency, or Bruton's agammaglobulinemia), μ-Heavy chain deficiency, l 5 deficiency, Igα deficiency, BLNK deficiency, thymoma with immunodeficiency
- B cells low but present or normal, but with reduction in 2 or more isotypes (usually IgG & IgA, sometimes IgM): common variable immunodeficiency (CVID), ICOS deficiency, CD19 deficiency, TACI (TNFRSF13B) deficiency, BAFF receptor deficiency.
- Normal numbers of B cells with decreased IgG and IgA and increased IgM: Hyper-IgM syndromes
- Normal numbers of B cells with isotype or light chain deficiencies: heavy chain deletions, kappa chain deficiency, isolated IgG subclass deficiency, IgA with IgG subsclass deficiency, selective immunoglobulin A deficiency
- Specific antibody deficiency to specific antigens with normal B cell and normal Ig concentrations
- Transient hypogammaglobulinemia of infancy (THI)
Secondary
Secondary (or acquired) forms of humoral immune deficiency are mainly:[3]
Presentation and complications
The signs and symptoms of humoral immune deficiency depend on the cause, but generally include signs of hypogammaglobulinemia (decrease of one or more types of antibodies) with presentations including repeated mild respiratory infections, and/or agammaglobulinemia (lack of all or most antibody production) which results in frequent severe infections and is often fatal.[4]
References
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- ↑ Lua error in package.lua at line 80: module 'strict' not found.
- ↑ Page 432, Chapter 22, Table 22.1 in: Lua error in package.lua at line 80: module 'strict' not found.
- ↑ Immunodeficiency by Dr. Saul Greenberg. University of Toronto. Last updated, on February 5, 2009
