Abstract
A 10 year old female has had repeated episodes of bacterial infections including pneumonia, otitis, adenitis, and urinary tract infections. She is short, has lymphadenopathy and a protuberant abdomen. IgG=1500 mg%, IgM=430 mg%, IgA= 68 mg%, IgD 18.5 mg% and IgE=l4 IU/ml. Ig Subclasses, inmunoelectrophoresis and complement component activity are normal. After isolating Group A streptococci from the throat and an excised node, ASO and other streptococcal antibody titers were low or undetectable. The excised lymph node showed reactive hyperplasia. Lymphocyte counts were normal. Blood is Group O and isoagglutinin titers (A and B) were absent. Responses to diphtheria, typhoid and hexavalent pneumococcal vaccine were minimal or absent. Normal numbers of B lymphocytes were determined by membrane immunofluorescence and rosettes. Spontaneous rosette formation with sheep erythrocytes was normal. In vivo delayed hypersensitivity was normal. Normal lymphocyte responses to mitogens and allogeneic cells occurred. Neutrophil (PMN) chemotaxis, mobility, phagocytosis and bactericidal activity were normal. A serum related defect in PMN opsonic and cidal activity with strep and E. coli was reversed with normal serum. The patient appears to represent a profound abnormality of antibody formation associated with recurrent infections.
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