hy·per·im·mu·no·glob·u·lin E syndrome (h  p r- my-n -gl b y-ln, --my -)n. An immunodeficiency disorder characterized by high plasma levels of a particular immunoglobin, a white blood cell chemotactic defect, and recurrent staphylococcal infections at various sites including the skin and upper respiratory tract. Also called Job's syndrome. |
hyperimmunoglobulin E syndrome
Hyper-IgE syndrome, Job syndrome Immunology An AR immunodeficiency characterized by chronic eczematoid lesions, recurring abscesses of skin, lungs, joints, recurring otitis media, sinusitis, life-threatening
staphylococcal infections, chronic eczemoid lesions and recurring abscesses of lungs, skin, joints, eyes, ears by Staph aureus, H influenzae, S pneumoniae, group A streptococci, Candida spp Lab ↑ Eosinophils, IgE > 5000 IU/mL,
↓ antibody response to vaccines and MHC antigens, ↓ CD4:CD8–T cell helper:suppressor ratio, defects in PMN and monocyte chemotaxis, defects in PMN and monocyte chemotaxis, ↑ IgE Prognosis Prolonged survival is possible
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