Pathology3 - I. Immune Disorders a. Inadequate = not...

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I. Immune Disorders a. Inadequate = not reacting immunodeficiency i. Can be antigen specific (adaptive) or non-specific (innate) ii. Everybody has to some extent (why there is more than one way to respond) iii. Functionally defect produced has to do with the point in the differentiation process at which the block occurred iv. Primary (1º) 1. disorders in which part of the body’s immune system is missing or does not function properly 2. inherited; gene defects 3. stem cell deficiency: lack of stem cells, or inability to drive stem cells (defect is before lymphoid cells are even processed) – die within first few weeks of life 4. Sever Combined Immunodeficiency Disease (SCIDs) – lack of enzyme (adenosine deaminase) to make successful antigen receptors (cells just become anergic) bubble kids a. Low Ig levels and lymphocyte count b. can’t produce antibodies of mount cell-mediated response c. most die within first few years of life 5. DiGeorge – no T cells (lack of thymus function); affects both cell mediated and humoral responses; inherited disorder 6. Bruton’s – B cells fail to mature (no antibodies; rely on innate response) a. B cell deficiency leads to repeated infection by pyogenic organisms b. Absent/low concentrations of Ig 7. IgA deficient (1/700 Caucasians); difficulty with respiratory infections, infection due to pyogenic organisms, but no other serious problems 8. hyper IgM 9. Macrophage deficient a. Failure to respond to chemotactic signals b. Failure of lysosomal fusion c. Failure of bacterial killing 10. Complement defects (C9 may never know due to other pathways; C3 would know pyogenic bacteria can’t be killed off) a. Absence of inhibitors (esp. C3 and C5) = bad v. Secondary (2º) 1. no evident defect in immune system but loss of immune function due to another cause (some kind of influence) 2. Age – system shut down (old); not responsive yet (young) 3. Nutrition – proteins, malnutrition 4. Neoplasm – lymphocytes killed off easily; shut down of other proteins needed 5. Iatrogenic – hospital acquired problems a. transplant must keep from reacting to organ, so suppress immune system b. therapeutics corticosteroids lower immune system; x=rays and cytotoxic drugs (chemotherapy)
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6. Infections (surgery or blood transfusion, immune system goes down; more susceptible); some viruses/bacteria sit in macrophages and therefore lower effectiveness of immune response 7. HIV a. Immune response: people kill off almost all of HIV; the rest hibernate for some time (immune response is actually great) b. Over time: CD4 cells getting killed off (Th1 and 2 included) c. Treatment: RNA constantly mutates; never any recognition because proteins it makes differ (drugs try to keep mutations down) d. AIDS can occur b. Inappropriate = overreacting Hypersensitivity i. Normal immune mechanisms and components ii. Over production and/or inappropriate targets iii. Type I IgE responds inappropriately (immediate, anaphylactic)
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This note was uploaded on 03/23/2009 for the course BLD 204 taught by Professor Gerlach during the Spring '09 term at Michigan State University.

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Pathology3 - I. Immune Disorders a. Inadequate = not...

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