Characteristic |
Predominant
T-Cell Defect |
Predominant
B-Cell Defect |
Granulocyte Defect |
Complement Defect
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Age at the onset of infection |
Early onset, usually 2-6 mo of age |
Onset after maternal antibodies diminish, usually after 5-7 mo of age, later childhood to adulthood |
Early onset |
Onset at any age |
Specific pathogens involved |
Bacteria: mycobacteria |
Bacteria: streptococci, staphylococci, Haemophilus, Campylobacter |
Bacteria: staphylococci, Pseudomonas, Serratia, Klebsiella |
Bacteria: Neisseria, Escherichia coli |
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Viruses: CMV, EBV, varicella, enterovirus |
Viruses: enterovirus (XLA) |
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Fungi and parasites: Candida; opportunistic infection, PCP |
Fungi and parasites: giardia, cryptosporidia |
Fungi and parasites: Candida; Nocardia, Aspergillus |
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Affected organs |
Failure to thrive, protracted diarrhea, extensive mucocutaneous candidiasis |
Recurrent sinopulmonary infections, chronic gastrointestinal symptoms, malabsorption, arthritis, enteroviral meningoencephalitis |
Skin: Dermatitis, impetigo, cellulitis |
Infections: meningitis, arthritis, septicemia, recurrent sinopulmonary infections |
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Lymph nodes: suppurative adenitis |
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Oral cavity: periodontitis, ulcers, internal organ abscesses, osteomyelitis |
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Special features |
Graft-versus-host disease caused by maternal engraftment or nonirradiated blood transfusion; Postvaccination, disseminated BCG or paralytic polio; hypocalcemic tetany in infancy (DiGeorge anomaly) |
Autoimmunity, lymphoreticular malignancy: lymphoma, thymoma; postvaccination paralytic polio |
Prolonged attachment of umbilical cord, poor wound healing |
Rheumatoid disorders: SLE, vasculitis, dermatomyositis, scleroderma, glomerulonephritis, angioedema |