Disease Information for Hypogammaglobulinemia/Agammaglobulinemia

Hypogammaglobulinemia is a disorder that is caused by a lack of B-lymphocytes and a resulting low level of immunglobulins (antibodies) in the blood; Immunoglobulins play a dual role in the immune response by recognizing foreign antigens and triggering a biological response that culminates in the elimination of the antigen; Antibody deficiency is associated with recurrent infections with specific types of bacteria; In pure B-cell disorders, cellular immunity generally is intact and the frequency of viral, fungal, and mycobacterial ( tuberculosis) infections is not increased; There are 5 major types of immunoglobulins: immunoglobulin G, immunoglobulin M (IgM), immunoglobulin A (IgA), immunoglobulin D (IgD), and immunoglobulin E (IgE); The most common congenital abnormalities of B lymphocyte production include:

Hypogammaglobulinemia (Common Variable Immunodeficiency)

Ig A Deficiency X-linked Agammaglobulinemia (Bruton Disease), Transient hypogammaglobulinemia of infancy,

Hypogammaglobulinemia or Common Variable Immunodeficiency (CVID); CVID is the second most common cause of hypogammaglobulinemia and affects both sexes equally; The incidence is about 1 in 50,000 people; In most patients there is a reduced amount of the immunoglobulins IgG, IgA, and IgM in the blood; It is an immune deficiency disorder that can be acquired or inherited; In most cases, there is no family history of immunodeficiency; However, in instances where more than one family member is affected, an autosomal recessive mode of inheritance is suggested; In about 5% of the cases, one or more of the family members can be found to be IgA deficient; IgA deficiency is the most common antibody deficiency syndrome, about 1 case in 700 persons; Recurrent infections may occur in as many as 50% of IgA-deficient patients, but most of these individuals are healthy; Recurrent or chronic upper and lower respiratory tract infections is common as is Giardia lamblia infections of the gastrointestinal tract; Patients with undetectable levels of IgA antibodies may develop severe allergic reactions if they receive blood products

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