Immunodeficiency in the Wiskott-Aldrich syndrome.
The immune system in the Wiskott-Aldrich syndrome is characterized by a host of paradoxes. Elements of the effector limbs of both humoral and cellular immunity such as immunoglobulin synthesis and in vitro lymphocyte proliferative, secretory and killer responses to nonspecific mitogens are intact. Yet, these patients fail to produce specific antibody normally following immunization and they are anergic. This combination of findings is consistent with a defect in the appropriate initiation of specific immune responses, ie a defect in the afferent limb of immunity.
D000373:Agglutinins; D000907:Antibodies, Bacterial; D000917:Antibody Formation; D000918:Antibody Specificity; D000946:Antigens, Fungal; D000956:Antigens, Viral; D001402:B-Lymphocytes / Q000276:immunology; D001419:Bacteria / Q000276:immunology; D001798:Blood Proteins / Q000378:metabolism; D002175:Candida / Q000276:immunology; D003601:Cytotoxicity Tests, Immunologic; D004168:Diphtheria Toxoid; D004912:Erythrocytes / Q000276:immunology; D004926:Escherichia coli / Q000276:immunology; D006433:Hemocyanins; D006460:Hemolysin Proteins; D007111:Immunity, Cellular; D007153:Immunologic Deficiency Syndromes / Q000209:etiology*; D008213:Lymphocyte Activation; D011135:Polysaccharides, Bacterial; D013296:Streptococcus pneumoniae / Q000276:immunology; D013299:Streptodornase and Streptokinase; D013301:Streptolysins; D013745:Tetanus Toxoid; D014923:Wiskott-Aldrich Syndrome / Q000150:complications* / Q000276:immunology
D000373:Agglutinins; D000907:Antibodies, Bacterial; D000946:Antigens, Fungal; D000956:Antigens, Viral; D001798:Blood Proteins; D004168:Diphtheria Toxoid; D006460:Hemolysin Proteins; D011135:Polysaccharides, Bacterial; D013301:Streptolysins; D013745:Tetanus Toxoid; D006433:Hemocyanins; D013299:Streptodornase and Streptokinase