Primary 'acquired hypogammaglobulinaemia': a suggested mode of inheritance

Abstract

The immune globulins of a patient having primary `acquired hypogammaglobulinaemia' and of his family were studied. Reduced serum concentrations of one or more factors were found in most family members.

Individual globulin synthesis is regulated by autosomal non-allelic genes. The recessive allele which manifests variable expressivity causes marked reduction of globulin synthesis in the homozygous state. `Acquired hypogammaglobulinaemia' occurs when synthesis of all three globulins is inhibited in the same individual.

A heterozygous carrier frequency of 1:5·6 for each globulin may be necessary to maintain `acquired hypogammaglobulinaemia' in the general population. In certain healthy family members the similarity of the immune globulin pattern to those found in patients having dysgammaglobulinaemia suggests that synthesis of other infection resistance factors besides the immune globulins may be impaired in the dysgammaglobulinaemias.