Hypogammaglobulinemia in a Young Girl

Abstract

Numerous reports of the association of hypogammaglobulinemia and frequent bacterial infections have appeared in the literature since Bruton's¹first report. Patients with this association have been classified as having (1) "transient hypogammaglobulinemia" in infants^2-5(1-6 months of age), which may be associated with severe infection⁶and sudden death⁷; (2) "congenital hypogammaglobulinemia," which appears to be familial and sex-linked and has been reported only in males^1-5,8-12; (3) "acquired hypogammaglobulinemia,"^13-37which has been reported in both sexes, mostly in adults but also in children; (4) "secondary hypogammaglobulinemia," idiopathic,^38,39due to urinary loss, as in nephrosis,^3-5or associated with diseases of the reticuloendothelial system,^40,41such as Hodgkin's disease and chronic leukemia; (5) "unclassified"—a group of patients which consists of girls whose symptoms have started in the first few years of life.^13-19,25The following case report has several unusual features that justifies reporting