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| Pregunta | Respuesta |
| 4 types primary deficiencies | 1. complement, 2. phagocytic, 3. humoral (B cell), 4. cellular (T cell) |
| C3 deficiency | prone to infections with encapsulated organisms. demonstrates how attachment of complement important for removal |
| examples of phagocytic defects (4) | 1. neutropenia, 2. LAD, 3. Chediak-Higashi, 4. CGD |
| neutropenia | too few neutrophils. result of stem cell differentiation failure |
| LAD (leukocyte adhesion deficiency) | defective CD18 req for adhesion to endothelium |
| Chediak-Higashi syndrome | mutation of LYST gene |
| CGD = chronic granulomatous disease | defect in genes encoding NADPH oxidase system involved in oxygen-dependent killing system |
| B cell deficiencies (3) | 1. CVID, 2. Bruton's, 3. Hyper IgM, |
| SCID- severe combined immunodeficiency | due to ADA/PNP or RAG deficiency |
| Bruton's agammaglobulinemia | congenital, x linked, defective tyrosine kinase gene |
| Hyper IgM syndrome | defects in CD40 gene, little or no IgG in system |
| T cell def: Di George syndrome | defect in thymus embryogenesis= incomplete thymus= decreased T cell numbers |
| T cell def: Wiscott Aldrich sydnrome | x linked, abnormal T cells due to chromosomal breaks in gene for T cell receptor |
| secondary immunodefs come from | infections, aging, drugs, trauma, malnutrition |
| factor: HIV | immunosupression by microbes |
| factor:malnutrition | lack of dietary elements inhibit lymphocyte function |
| factor: tumours | immunosupressive molecules eg TGFbeta |
| factor: cytotoxic drugs | chemo |
| factor: aging | decreased quality of cells and responses |
| HIV infects | helper T cells |
| treatment of CGD | 1. bone marrow transplant 2. antibiotics |
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