PATHOLOGY OF THE IMMUNE RESPONSE 



In a very small proportion no formation of gamma globulin 

 ever occurs, presumably as a result of a genetic defect 

 (Bruton, 1952). Such cases of congenital agammaglobulin- 

 aemia are of very great interest for immunological theory. 



The condition, at least in typical form, is seen only in 

 males and appears to be a sex-linked genetic anomaly. The 

 diagnostic feature is an absence of demonstrable gamma 

 globulin and of circulating antibody of all types. According 

 to Martin (1954), the principal defect is in the y^ globulin. 

 There are no natural z^oagglutinins and no serological 

 responses to any of the standard immunizing antigens. 



Clinically, the usual feature is undue susceptibility to 

 pyogenic infection, especially of the respiratory tract. Other- 

 wise the children appear normal. It is of particular interest 

 that they show normal responses to the common virus 

 diseases, measles, chicken-pox and polio, influenza and Jen- 

 nerian vaccination. In Bruton's first case three attacks of 

 mumps were reported, but subsequent authors have found 

 normal immunity to follow the primary attack and it appears 

 highly probable that in this instance the subsequent attacks 

 were of bacterial parotitis. In general, these virus infections are 

 clinically normal and are followed by typical solid immunity. 



Histologically the only characteristic feature is absence 

 of plasma cells in the bone marrow and in lymph nodes 

 draining areas that have received immunizing injections. 



Other immunological features of interest are in relation to 

 the responses which are not mediated by circulating anti- 

 body. The first report of skin transplantation (Good and 

 Varco, 1 955 3) indicated that a graft from an unrelated 

 individual was accepted for a long period, but subsequent 

 experience indicates that all homografts are eventually 

 rejected. Patients can be actively sensitized to tuberculin 

 by BGG immunization (Porter, 1955) and to 2-4-dinitro- 

 fluorobenzene by treatment of the skin with the allergen. 

 Sensitivity to the latter can be transferred passively to normal 

 recipients by blood leucocytes (Good and Zak, 1956). 



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