A 15-year-old boy with x-linked agammaglobulinemia (X-LA) was evaluated for growth retardation, malabsorption and progressive inflammatory bowel disease. He had absent IgG, IgM and IgA by immunoelectrophoresis. Surface immunoglobulins (Ig) were absent by polyvalent and IgM antisera. EAC-rosette forming lymphocytes were decreased (6% vs. 14% control). E-rosette forming lymphocytes were near normal (47% vs. 61% control). PHA, ConA and PWM-stimulation were normal. Response to skin tests with Mumps and SKSD were normal; monilial antigen were nonreactive. Small bowel series revealed fixed, nodular ileum with widely separated loops. Rectal biopsy showed “nests” of lymphocytes. There were no plasma cells or detectable Ig's by immunofluorescent staining. Fecal fat excretion (coef.=82), infused IgG half-life (4 days) and Schilling Test (0% excretion) were abnormal. Laparotomy was performed and revealed inflammatory, polypoid-like ileal mucosa. There was massive infiltration of the mucosa and submucosa with mature lymphocytes. Polypoid-like lesions represented masses of lymphocytes. There were no detectable tissue Ig's as determined by single phase antibody radioimmunoassay. E-rosettes forming intestinal lymphocytes were 51%; EAC-rosetting lymphocytes were 5%. No virus was isolated from surgical tissue. This case appears to represent a new, unclassified inflammatory bowel disease of unknown etiology in a child with X-LA.
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Halpin, T., Polmar, S., Izant, R. et al. 429 X-LINKED AGAMMAGLOBULINEMIA AND INFLAMMATORY BOWEL DISEASE. Pediatr Res 12, 435 (1978). https://doi.org/10.1203/00006450-197804001-00434