Antibody deficiency syndromes and novel immunodeficiencies

Antibody deficiency syndromes can be quantitative or qualitative. The major categories of antibody deficiency syndrome are: (1) X-linked agammaglobulinemia, involving the maturation arrest in the development of the B cells; (2) transient agammaglobulinemia, which affects both sexes is often associated with defective T helper function for immunoglobulin production; (3) acquired agammaglobulinemia, a heterogeneous disorder caused by a primary B cell defect, absence of B cells, presence of B cells but with an activation defect, failure of helper factor production by T cells or increase in suppressor cells; (4) IgG2 and IgG3 subclass deficiencies, causing significant and recurrent infections and, with IgG2, a significant impairment of the ability to respond to carbohydrate antigens such as Haemophilus influenzae, pneumococcus and meningococcus; (5) qualitative antibody deficiency syndrome in the response to carbohydrate antigens, presenting as recurrent infection and involving the inability of the patient to respond to immunization with polysaccharide antigens such as Haemophilus influenzae type b; (6) antibody deficiency states associated with T cell dysfunction. Impairment of T cell function, which is required for B cell activation, presents often as antibody deficiency syndrome. In these cases, total gamma-globulin level is normal, but the quality of the antibody is very poor.(ABSTRACT TRUNCATED AT 250 WORDS)