A 13 yr old male with selective IgA deficiency, a flat jejunal mucosa, and severe, fatal malabsorption is reported. Unlike previously well described cases, there was no response to a gluten free diet, and multiple forms of therapy were not helpful. The mucosal lesion was the only basis demonstrated for malabsorption, and contributing factors, such as giardiasis and bacterial overgrowth, were not present. Infusions of fresh frozen plasma raised the serum IgA from undetectable levels to 14.5 to 16.0 mg/100 ml, but there was no obvious beneficial effect from this. Investigations of the jejunal mucosa demonstrated proliferation of plasma cells and an increased proliferation of epithelial cells as in gluten sensitive enteropathy. This case illustrates that in the occasional patient who initially appears to have gluten enteropathy, but does not respond to treatment, correction of coexisting selective IgA deficiency by plasma infusions may not be helpful.
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