hyperimmunoglobulin E syndrome


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hy·per·im·mu·no·glob·u·lin E syn·drome

an immunodeficiency disorder characterized by high levels of plasma IgE concentrations, a leukocyte chemotactic defect, and recurrent staphylococcal infections, and cold-abscess formatin in the skin, upper respiratory tract, and other sites.
Synonym(s): Job syndrome
An immunodeficiency characterised by multiple recurring abscesses
Lab Defects in neutrophil and monocyte chemotaxis, increased IgE

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

hy·per·im·mu·no·glob·u·lin E syn·drome

(hī'pĕr-im'yū-nō-glob'yū-lin sin'drōm)
An immunodeficiency disorder characterized by high levels of plasma IgE concentrations, a leukocyte chemotactic defect, and recurrent staphylococcal infections of the skin, upper respiratory tract, and other sites.
References in periodicals archive ?
Hyperimmunoglobulin E syndrome (HIES) is a primary immunodeficiency disorder characterised by recurrent sino-pulmonary infections, cutaneous abscesses and chronic eczematous dermatitis.
Clinical and immunologic aspects of hyperimmunoglobulin E syndrome.