immunodef 2

Description

Immunopathology Flashcards on immunodef 2, created by lumen7 on 07/05/2013.
lumen7
Flashcards by lumen7, updated more than 1 year ago
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Created by lumen7 over 11 years ago
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Resource summary

Question Answer
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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