In 1952 Bruton1 reported the first known case of agammaglobulinemia. With newer refinements in methods of analysis of serum proteins, a clearer concept of this syndrome has developed.
In brief, the clinical features of agammaglobulinemia or hypogammaglobulinemia are:
Extreme susceptibility to infection
Low to absent γ-globulin in the serum
Immunologic unresponsiveness to antigenic challenge
Frequent clinical improvement of symptoms with γ-globulin therapy.
Physical examination usually reveals no abnormalities, although there may be a generalized decrease in lymphoid tissue and lack of regional lymphadenopathy in response to local infection.2
Because of the lack of specificity of signs and symptoms, only a high index of suspicion and the use of appropriate laboratory studies can lead to the correct diagnosis. On routine determination of total serum proteins a globulin of 1 gm. % or less is suggestive of hypogammaglobulinemia. The diagnosis can be confirmed by serum electrophoresis or immunochemical analysis. Other laboratory findings