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Immunology day 2

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Question
Answer
Which antibodies can be given passively?   To Be Healed Rapidly: Tetanus, Botulinum, HBV, RabiesI  
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Isotype switching is mediated by   CD40 ligan, IL-4, IL-5, Il-6  
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IgA functions   Prevents attachment of bacteria and viruses to mucous membranes, does not fix complement  
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Defect in tyrosine kinase gene (BTK). Name disorder, what you would see, lab findings, inheritance?   (decreased production) Bruton's agammaglobinulinemia. Recurrent bacterial infections after 6 month (maternal IgG levels decline). Low B cells, normal pro-B cells. X linked  
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Thymus and parathyroids fail to developt owing to failure of development of 3/4 pouch. Name disorder, what you would see, lab findings, inheritance   (Decreased production). CATCH-22 (Cardiac defects, abnromal facies, thymic hypoplasia, cleft palate, hypocalcemia (tetany), microdeletion of 22). Will have recurrent fungal/viral infections. 22q11 deletion  
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Name the multiple causes of SCID and labs for each.   Causes: Failure to synthesize MHC II (noraml immature T cells), defective IL-2 receptor (normal Pro-T cells, X-linked), ADA deficiency (only stem cells present)  
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No rejection of allografts   SCID  
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Patient with SCID will present:   Recurrent viral, bacterial, fungal, protozoal infections. No rejection of allografts, high incidence of malignancy, GVH disease, FTT)  
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Decreased activation of T cells is a result of what deficiency? What cells are deficient?   IL-12; causes disseminated mycobacterial infections due to decreased TH1 resposne  
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decreased activation of B cells results in what disorder? presentation, lab values   hyper-IgM syndrome; CD40L on CD4 T helper cells --> inability to class switch. Presents with pyogenic infections. High IgM, low IgG, IgE, IgA  
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Disorder= high IgM, low IgG, IgE, IgA, Disorder= high IgE, IgA, low IgM, Disorder = High IgE   Hyper-IgM syndrome, Wiskott-Aldrich syndrome, Job's syndrome  
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Defect in the ability to mount an IgM response to capsular polysaccharides of bacteria. Disorder, mechanism, presentation, lab, genetics   Wiskott-Aldrich syndrome (decreased activation of B cells), "WIPE" = pyogenic Infections, thrombocytopenic Purpura, Eczema, high IgE, IgA, X-linked  
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Neutrophils fail to respond to chemotactic stimuli   Job's syndrome (decreased activity of neutrophils), Failure of IFN-gamma production by helper T cells. (FATED) Coarse facies, cold staphylococcal abcesses, retained primary Teeth, high igE, Dermatologic problems (eczema)  
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Defect in LFA-1integrin (CD_) proteins on ___   CD18, phagocytes; LAD defciency (phagocyte cell deficiency). Early recurrent bacterial infections, absetn pus formation, neutrophila, delayed seperation from umbilical cord  
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Defect in microtubular function and lysosomal emptying of phagocytic cells. Presentation, genetics   Chediak-Higashi (phagocytic cell deficiency). Recurrent pyogenic infections, by staph, strep, partial albinism, peripheral neuropathy, autosomal recessive  
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Defect in microbiocidal activity of ____. Describe presentation. Describe diagnostic test   NADPH oxidase. Chronic granulomatous disease (deficiency of phagocytes). Presents with marked susceptibility to S. aureus, E. coli, Aspergilllus. Negative nitroblue tetrazolium dye reduction test  
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Chronic mucotaneous candidiasis is a dysfunction in what cell type?   T cell dysfunction against candida. Presents with skin and mucous membrane candida infections  
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Deficiency in a class of immunoglobulins   Selective immunoglobulin deficiency. Selective IgA deficiency is the most common; Presents with sinus, lung infections, milk allergies, diarrhea.  
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milk allergies, diarrhea due to -   IgA deficiency  
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Anaphylaxis to bloo products   IgA deficiency  
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Defect in DNA reapir enzymes. Its associated with what deficiency? presents with what problems?   Ataxia telangiectasia; associated with IgA deficiency. cerebellar problems, spider angiomas (due to ATM gene) = autosomal recessive, tumor suppressor  
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decreased plasma cells but normal B cells   Common variable immunodeficiency; present in 20s-30s; increased risk of autoimmune disease, lymphoma.  
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Bugs that have antigenic variation include   salmonella (flagellar), borrelia, Neisseria (pilus), Virus (shift=rearrangment, drift=random mutation), trypanosomes (african sleeping sickeness)- programmed rearrangement  
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Anergy:   self-reactive molecules become non-reactive with costimulatory molecule  
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Types that have granulomatous disease   Tuberculosis, Fungal (histo), syphillis, leprosy, Cat scratch, sarcoid, crohn's, berylliosis (non-caseating) (aerospace/nuclear industry)  
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Define granuloma   Nodular collection of specialized macrophages (epitheliod cells) surrounded by a rim of lymphocytes)  
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Desribe progression of granuloma   APC eats antigen; presents on MHC II--> matched with CD4; TH1 stimulates IFN gamma--> monocyte --> macrophage--> epitheliod cell --> giant cell  
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Components of granuloma   epitheliod cell, giant cell, fibroblasts, lymphocytes.  
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Difference between Langhans cells and foreign body   Langhans= multiple nuclei in horseshoe conformation; Foreign body = scattered nuclei  
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Radioimmunosorbent test   Type I (detects specific igE antibodies in serum agasint specific allergies)  
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Coomb's: Direct/Indirect; hypersensitivity __   Direct: detects IgG/C3b attached to RBC; Indirect: detects antibodies in serum (Rh anti-D); II  
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Immunofluoresecnt staining   III  
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Alternative pathway is part of ___ immune system. Eg:   LPS, gram -; innate  
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Classical pathway is part of ___ immune system; it involves   C1-C4 = viral neutralization; adaptive  
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Mannose binding lectin pathway   Used for binding to sugars on surface  
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C3b =, c3a = c5a =   C3b = opsonization, c5a, c3a = anaphylotoxins, c5a = neutrophil chemotaxis  
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Deficiency of c1 esterase leads to   hereditary angioedema = problem with classical system; C1 esterase= noramlly controls complement; without it you'll have a decrease in C2-C4, increased cleavage products --> anaphylaxis (swelling of face/oropharynx)  
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C2 deficiency   Association with septicemia (S. pneumoniae), lupus like syndrome in cildren  
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C6-C9/C5-8 deficiency   Increased susceptibility to Neisseria/mengitidis  
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DAF function   (decay accelerating factor): prevents complemetn activation of self cells  
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DAF deficiency   due to a defect in the the anchoring of the particle; leads to complement mediated lysis for RBCs and paroxysmal nocturnal hemoglboinuria (platelets, neutrophils, hemoglobinuria)  
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Function of interferons:   induce production of ribonuclease that inhibits viral protein synthesis by degrading viral mRNA. Also activates NK cells to kill virus-infected cells  
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Alpha/beta interferons ___-, while gammma does ___   alpha/beta = inhibit viral protein synthesis, gamma= increases MHC I/II expression and antigen presentation on cells  
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