Abstrakt: |
Numerous reports of the association of hypogammaglobulinemia and frequent bacterial infections have appeared in the literature since Bruton's1 first report. Patients with this association have been classified as having (1) "transient hypogammaglobulinemia" in infants2-5 (1-6 months of age), which may be associated with severe infection6 and sudden death7; (2) "congenital hypogammaglobulinemia," which appears to be familial and sex-linked and has been reported only in males1-5,8-12; (3) "acquired hypogammaglobulinemia,"13-37 which has been reported in both sexes, mostly in adults but also in children; (4) "secondary hypogammaglobulinemia," idiopathic,38,39 due to urinary loss, as in nephrosis,3-5 or associated with diseases of the reticuloendothelial system,40,41 such 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.13-19,25 The following case report has several unusual features that justifies reporting |