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. 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.

Signs and symptoms

Signs/symptoms of humoral immune deficiency depend on the cause, but generally include signs of infection such as: *

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Sepsis Sepsis, formerly known as septicemia (septicaemia in British English) or blood poisoning, is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage is follo ...

Skin infection A skin infection is an infection of the skin in humans and other animals, that can also affect the associated soft tissues such as loose connective tissue and mucous membranes. They comprise a category of infections termed skin and skin structur ...

Pneumonia Pneumonia is an inflammatory condition of the lung primarily affecting the small air sacs known as alveoli. Symptoms typically include some combination of productive or dry cough, chest pain, fever, and difficulty breathing. The severi ...

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: 170 px, Hyper-IgM syndromes(immunoglobulin M) :* Absent B cells with a resultant severe reduction of all types of antibody: X-linked agammaglobulinemia ( btk deficiency, or

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'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 Immunoglobulin M (IgM) is one of several isotypes of antibody (also known as immunoglobulin) that are produced by vertebrates. IgM is the largest antibody, and it is the first antibody to appear in the response to initial exposure to an antig ...

: 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:Page 432
Chapter 22, Table 22.1 in: :* Multiple myeloma :* Chronic lymphoid leukemia :* AIDS

Diagnosis

In terms of diagnosis of ''humoral immune deficiency'' depends upon the following: * 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: * Treatment of

infection An infection is the invasion of tissues by pathogens, their multiplication, and the reaction of host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmissible disease or communicable dis ...

(antibiotics) * Surveillance for malignancies * Immunoglobulin replacement therapy

References

External links

PubMed
{{Medicine Predominantly antibody deficiencies