Selective hypogammaglobulinemia with persistence of IgE, malabsorption and a nutritionally dependent, reversible defect in cell-mediated immunity.
A 46 year old man presented with selective hypogammaglobulinemia, malabsorption and long-standing secondary malnutrition. Although the patient had essentially unmeasurable levels of immunoglobulins G (IgG), M (IgA), he had normal levels of immunoglobulin E (IgE). He was found to be anergic when tested for the delayed cutaneous hypersensitivity reaction. Evaluation of his cell-mediated immunity in vivo and in vitro suggested one discrete lesion, a defective production of the lymphocyte mediator macrophage migration inhibitory factor. With improved nutrition the patient "repaired" this defect in the "efferent" limb of cellular immunity and was no longer anergic.
D000361:Agammaglobulinemia / Q000276:immunology*; D004032:Diet; D006801:Humans; D007111:Immunity, Cellular; D007070:Immunoglobulin A / Q000032:analysis; D007073:Immunoglobulin E / Q000032:analysis*; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D007413:Intestinal Mucosa / Q000276:immunology; D008263:Macrophage Migration-Inhibitory Factors / Q000172:deficiency; D008264:Macrophages / Q000276:immunology; D008286:Malabsorption Syndromes / Q000209:etiology / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009748:Nutrition Disorders / Q000209:etiology / Q000276:immunology; D012882:Skin Tests
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D008263:Macrophage Migration-Inhibitory Factors; D007073:Immunoglobulin E