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, or the antibody secreted by the plasma cells.[7] The most common such immunodeficiency is inherited selective IgA deficiency, occurring between 1 in 100 and 1 in 1000 persons, depending on population. They are associated with increased vulnerability to infection, but can be difficult to detect (or asymptomatic) in the absence of infection.[citation needed]
Signs and symptoms
Signs/symptoms of humoral immune deficiency depend on the cause, but generally include signs of infection such as:[1]
Causes
Cause of this deficiency is divided into primary and secondary:
- Primary the International Union of Immunological Societies classifies primary immune deficiencies of the humoral system as follows:[3][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, 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
- Transient hypogammaglobulinemia of infancy (THI)
- Secondary secondary (or acquired) forms of humoral immune deficiency are mainly due to hematopoietic malignancies and infections that disrupt the immune system:[4]
Diagnosis
In terms of diagnosis of humoral immune deficiency depends upon the following:[5][6]
- Measure serum immunoglobulin levels
- B cell count
- Family medical history
Treatment
Treatment for B cell deficiency (humoral immune deficiency) depends on the cause, however generally the following applies:[5]
- Treatment of infection (antibiotics)
- Surveillance for malignancies
- Immunoglobulin replacement therapy
See also
References
Further reading
External links
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