Job syndrome is due to
The autosomal dominant hyper-IgE syndrome also known as Job syndrome, characterized by the clinical triad of high serum IgE, recurring staphylococcal skin abscesses, and recurrent pneumonia with the formation of pneumatoceles.There is a defective immune response involving T lymphocytes, neutrophils and the cytokines they produce, especially interferon-gamma. Excessive levels of interferon-gamma result in marked elevation of immunoglobulin E.Disorders of phagocytosis:1.Chronic granulomatous disease2.Myeloperoxidase deficiency3.Chediak-Higashi syndrome4.Leukocyte G6PD deficiency5.Job's syndrome6.Tuftsin deficiency7.Lazy leukocyte syndrome8.Hyper- IgE syndrome9.Actin-binding protein deficiency10. Shwachman's diseaseRef: Ananthanarayan 9th edition, p172
Defect in chemotaxis
Defect in phagocytosis
Defect in synthesis
Defect in leukocyte function
Immunology