B cell deficiency

Humoral immune deficiency
B cells and antibody
Classification and external resources
Specialty	hematology
ICD-10	D80
ICD-9-CM	279.0
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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. 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.

They can be associated with increased risk of gastric cancer.

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.

One way of classifying humoral immune deficiencies is into primary (or hereditary) versus secondary (or acquired) ones.

The International Union of Immunological Societies classifies primary immune deficiencies of the humoral system (here termed "Predominantly antibody deficiencies") as follows:

Secondary (or acquired) forms of humoral immune deficiency are mainly due to hematopoietic malignancies and infections that disrupt the immune system:

In most cases, treatment consists of avoiding infection and managing infections with antibiotics or antivirals. In case of life-threatening diseases, bone marrow transplant may be attempted.

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Wikipedia