Frage | Antworten |
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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