CONGENITAL AGAMM AGLOBULINAEMIA 



The absence of natural 2.yoagglutinins speaks strongly for 

 the view that 'normal' antibodies are closely related to 

 classical acquired antibodies. Good and Varco (1955^) have 

 shown that even when patients are injected with foreign 

 erythrocytes which in normal persons of their blood group 

 would provoke an active rise in titre, there is no response 

 whatever. 



Rohn, Behnke and Bond (1954) describe a patient with 

 agammaglobulinaemia who developed idiopathic haemolytic 

 anaemia without evidence of auto-antibody production. The 

 condition was cured by splenectomy ; histologically the spleen 

 showed excessive erythrophagocytosis. 



These findings seem to be much more readily co-ordinated 

 on a basis of the clonal selection theory than of any other 

 theoretical concept. The basic anomaly on this view is a 

 failure of lymphocytes (or other mesenchymal cells) carrying 

 specific antibody-producing potentialities to develop into 

 plasma cells, which are the only producers of circulating 

 antibody. The functions that can be ascribed to cell-borne 

 antibody are carried out normally or with only somewhat 

 less efficiency than in the normal individual. 



The three features of special interest are (i) the normal 

 course of infection and immunity in virus disease, (ii) the fact 

 that haemolytic anaemia can occur in an agammaglobu- 

 linaemic child, and (iii) the common occurrence of arthritis 

 of rheumatoid type in children who have been maintained in 

 reasonable health for some years by appropriate therapy. 

 Each of these could be used as a text for an extensive dis- 

 cussion and as a stimulus to new experimental work on the 

 phenomenon in question. Here we shall attempt only a very 

 brief discussion to show the relation of these observations 

 to the clonal selection theory. 



(i) The virus diseases which have been reported on are all 

 conditions of long incubation period in which there is reason 

 to believe that the virus may spend the incubation period in 

 intimate relationship with the lymphoid tissues, cf the Warthin 



143 



