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131 Cards in this Set
- Front
- Back
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what is the B cell receptor for EBV?
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CD21
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what type of cell as CD56?
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NK cell
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what kind of cell expresses CD14?
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macrophage
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what kinds of cells express B7?
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APCs: B cells, macrophages, dendritic cells (this is signal 2 for Th cells)
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what kinds of cells express CD3
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all T cells - these are proteins assoc w TCRs
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what types of cells do NOT express MHC 1?
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MATURE RBCs
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what kind of cell expresses CD16?
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NK
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what kind of cell expresses CD40L?
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Th cell - this is signal 2 for activating a B cell
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what kind of cell expresses CD28?
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helper T cell - this is what binds to B7 in signal 2 to activate the Th cell
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what kind of cell has receptors for C3b?
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macrophages
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what are the 2 primary opsonins in bacterial defense?
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IgG and C3b
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what is the function of DAF and C1 esterase?
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prevent complement activation on self cells
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what complement component aids in clearance of immune complexes?
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C3b
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which complement pathway involves C4?
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Classic, Lectin
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What is the c3 convertase for the alternative pathway?
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C3bBb
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what complemet pathway involves factor B & D?
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alternative
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what is the trigger for the alternative complement pathway?
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nonspecific microbial surfaces, ie, endotoxin
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what is the C3 convertase for the classic pathway?
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C4b2b
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what is the C5 convertase for the aternative complement pathway?
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C3bBbC3b
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what is the C5 convertase for the classic complement pathway?
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C4b2a3b
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what 2 products released from the complement cascade cause anaphylaxis?
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C3a & C5a
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what product released from the complement cascade causes neutrophil chemotaxis?
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C5a (5 neutrophils is MORE than 3 - that's how you remember C5a over c3a)
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C1 esterase deficiency can cause:
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hereditary angioedema
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C3 deficiency can cause?
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severe recurrent pyogenic sinus & respiratory tract infxns, encapsulated bacteria (Salmonella, H flu, S Pneumo, N meningitidis; also suscepyibility to type III hypersensitivity rxns
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deficiency in C5-C8 causes what types of infxns?
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Neisseria
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DAF deficiency causes:
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complement mediated lysis of RBCs -> paroxysmal nocturnal hemoglobinuria
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what do alpha and beta interferons do? (mechanism)
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induce production of a ribonuclease that inhibits viral protein synthesis (degrade viral mRNA; activate NK cells
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what is the mechanism of gamma interferon
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increase MHC II expression and antigen presentation in all cells; activate NK cells
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list 4 viruses where passive immunity (vaccines) are administered to 'heal rapidly'
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Tetanus toxin, Botulinum toxin, HBV, Rabies
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list 3 bacteria which display antigenic variation in the course of an infection
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Neisseria Gonorrhea (pilus), Salmonella (2 flagella), Borrelia (relapsing fever)
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list a virus which can quickly undergo antigenic variation
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influenza
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list a parasite which is known for antigenic variation
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trypanosomes (african sleeping sickness, Chagas)
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granulomatous disease is a Th1 or Th2 mediated process?
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Th1 - secretes lots of IFN-gamma; intracellular processes
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List 8 diseases known for forming granulomas
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TB, sarcoid, fungal (histo), Syphilis, Leprosy, Cat Scratch Fever, Berylliosis (+ other occupational lung dz), Crohn's
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type I hypersensitivity rxns are caused by?
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free antigen cross links w pre-formed (sensitized) IgE on mast cell/basophil -> atopic & anaphylactic
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2 tests for type I hypersensitivity rxn
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scratch test and radioimmunosorbent assay (RAST test)
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type II hypersensitivity
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antibody mediated, 'cytotoxic' - IgM or IgE binds to fixed enemy antigen -> complement or phagocytosis; recruit neutrophils/macrophages -> tissue destruction; bind to other tissues
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test for type II hypersensitivity
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direct & indirect coombs
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type III hypersensitivity
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immune complexes in the serum -> attract neutrophils -> release lysosomal enzymes
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what causes serum sickness, what type of hsnsitivity is it, & how long does it take to come
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type III hypersensitivity, immune complexes deposit on membranes & fix complement; mostly caused by drugs, 5-10 days post antigen exposure
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what is an Arthus rxn?
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local, subacute type 3 hsnsitivity; example - intradermal injection of an antigen that then induces antibodies, producing immune complexes in the SKIN - edemam necrosis, activation of complement
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test for type 3 hsensitivity rxn?
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immunofluorescent staining
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type IV hypersensitivity rxn?
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delayed, cell-mediated - sensitized T cells kill
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TB skin test is a type ___ hypersensitivity rxn
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IV
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contact dermatitis is a type ____ hypersensitivity rxn?
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IV
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multiple sclerosis is a type ____ hypersensitivity rxn?
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IV
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Hemolytic Anemia is a type ____ hypersensitivity rxn?
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II
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SLE is a type ____ hypersensitivity rxn?
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III
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Guillan Barre is a type ____ hypersensitivity rxn?
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IV
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Pernicious Anemia is a type ____ hypersensitivity rxn?
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II
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Rheumatoid Arthritis is a type ____ hypersensitivity rxn?
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III
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Hashimoto's Thyroiditis is a type ____ hypersensitivity rxn?
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IV
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ITP is a type ____ hypersensitivity rxn?
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II
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Polyarteritis Nodosa is a type ____ hypersensitivity rxn?
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Reveiw: what is this?
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III; Kussmaul's, a medium sized artery vasculitis
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Erythroblastosis fatalis is a type ____ hypersensitivity rxn?
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II
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Graft-vs-Host disease is a type ____ hypersensitivity rxn?
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IV
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Rheumatic Fever is a type ____ hypersensitivity rxn?
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II
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Hypersensitivity Pneumonitis (farmer's lung)is a type ____ hypersensitivity rxn?
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III
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Goodpasture's is a type ____ hypersensitivity rxn?
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II
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Bullous Pemphigoid is a type ____ hypersensitivity rxn?
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II
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Pemphigus Vulgaris is a type ____ hypersensitivity rxn?
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II
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Graves is a type ____ hypersensitivity rxn?
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II
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Myesthenia Gravis is a type ____ hypersensitivity rxn?
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II
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Type I DM is a type ____ hypersensitivity rxn?
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IV
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swelling after a tetanus shot is an example of a _____ rxn and is a type ____ hypersensitivity rxn?
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Arthus, III
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Alopecia is associated with what HLA type?
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DQ3
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what gene is defective in Bruton's agammagloinuria? what is the effect?
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BTK, a tyrosine kinase, blocking B cell differentiation & maturation. After maternal IgG decreases (6 mos) baby gets recurrent bacterial infxns
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inheritance pattern of Bruton's agammaglobinuria?
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X linked recessive - only affects boys
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Hyper-IgM syndrome is caused by a defective _____ that causes what?
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CD40Ligand on helper T cells -> prevents class switching -> severe pyogenic infetions early in life
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What Ig is most commonly deficient in selective Ig deficiency, and what is the presentation?
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defective isotype switching; IgA; sinus & lung infections, milk allergies, diarrhea, anaphylactic rxn if exposed to IgA containing blood products
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what is defective in CVID (common variable immunodeficiency) and when do you get this?
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B cell maturation; can be acquired in 20s & 30s -> risk of autoimmune diseases, lymphoma, sinopulmonary infxns
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what would the labs look like for someone with CVID (# B cells, plasma cells?)
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normal B cells but low plasma cells & low immunoglobulins
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what gene is deleted in thymic aplasia?
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22q11 - failure to develop 3rd & 4th phryngeal pouches
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What is the consequence of DiGeorge syndrome?
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low T cells, no thymus, heart defects, recurent viral & fungal infxns, hypocalcemia (tetany)
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What is the consequence of IL-12 receptor deficiency? what are the IFN-gamma levels?
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low Th1 response -> disseminated mycobacterial infections; low IFN-gamma
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what is another name for Hyper-IgE syndrome?
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Job's syndrome
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what is the pathology of Job's syndrome?
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Th cells fail to make IFN-gamma -> neutrophils cannot respond to chemotactive stimuli
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what does a patient with Job's syndrome present with?
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high IgE, red hair, course face, dermatitis, retained primary teeth, 'cold' staphylococcal abscesses
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someone with chronic mucosal candidiasis has a dysfunction in ___ cells
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T cells
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what is the most common type of SCID? (defect and inheritance pattern)
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defective IL-2 receptor, usually X linked
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adenosine deaminase deficiency can cause what immune disorder?
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SCID
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failure to synthesize MHC antigens can cause what immune disorder?
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SCID
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treatment of SCID
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bone marrow transplant
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impairment in enzymes that ______ causes ataxia telangectasia
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repair DNA
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pt presentation (triad) in ataxia telangectasia
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cerebellar (ataxia); spider angiomas; IgA deficiency
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what is progressively deleted in Wiskott Aldrich syndrome
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?
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B & T cells
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what is the inheritance patter of Wiskott Aldrich syndrome?
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X linked
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What is the triad (presentation) of Wiskott Aldrich syndrome?
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TTP, Infections, Eczema (TIE - boys)
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what Ig's are high and what Ig's are low in Wiskott-Aldrich syndrome?
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hi IgE and IgA; low IgM
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What is the protein defect in Leukocyte Adhesion Deficiency type I?
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LFA-1 integrin (CD18 protein) on phagocytes
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in what immune disorder might you see delayed separation of the umbilicus?
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Leukocyte Adhesion Deficiency type I
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absent pus formation is seen in what immune disease?
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Leukocyte Adhesion Deficiency type I
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defective phagolysosome formation is seen in what disease?
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Chediak Higashi syndrome
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partial albinism can be seen in what disease?
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Chediak Higashi syndrome
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Chediak Higashi syndrome inheritance pattern?
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AR
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defective microtubule formation/affects release of lytic granules
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Chediak Higashi syndrome
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Lack of NADPH oxidase
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Chronic Granulomatous disease
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Susceptibility to what organism is seen in CGD?
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catalase positive organisms (S. Aureus, E. Coli, Aspergillus)
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test for CGD
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Nitroblue tetrazolium dye reduction test is negative (respiratory/oxidative burst turns it blue)
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inheritance pattern of CGD
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X linked recessive
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what is a syngeneic graft?
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a graft from an identical twin
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hyperacute rejection of a graft is a type ___ hypersensitivity that occurs in ___(amount of time____ after transplant and is caused by ______
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II; within minutes; anti-donor antibodies from the graft
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when does acute rejection of a graft occur and what is the mechanism'?
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within a few weeks; cell mediated due to cytotoxic T cells reacting against foreign MHC
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list 2 immunosuppressant drugs that can reverse acute rejection?
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cyclosporine & OKT3
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what is the mechanism of chronic rejection? is it reversible?
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irreversible; T cell and antibody mediated vascular damage, fibrosis; class I MHC from non self are perceived by CTLs as self MHC-I presenting a foreign antigen
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what is graft versus host disease? What are 4 physical manifestations?
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immunocompetent T cells from the GRAFT DONOR react against the new host's tissues - maculopapular rash, jaundice, hepatosplenomegaly, diarrhea
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what is the mechanism of cyclosporine?
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binds to cyclophilins-> that complex inhibits calcineurin -> cant phosphorilate NFAT -> prevents IL-2 production -> suppresses T cells
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what is a toxicity of cyclosporine?
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nephrotoxicity
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what is cyclosporine indicate for?
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suppression of organ rejection after transplant; autoimmune diseases
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what is the mechanism of tacrolimus?
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binds with FK-binding protein -> that complex inhibits calcineurin -> cant phosphorilate NFAT -> prevents IL-2 production -> suppresses T cells
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what is more toxic, tacrolimus or cyclosporine?
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tacrolimus - nephrotoxicity, peripheral neuropathy, HTN, pleural effusion, and hyperglycemia
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indication for tacrolimus?
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organ transplant immunosuppressant
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mechanism of azathioprine
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antimetabolite, precursor to 6-mercaptopurine -> intereferes w DNA synthesis -> blocks proliferating lymphocytes
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Clincal use of azathioprine
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kidney transplant, autoimmune glomerulonephritis, AIHA, + other autoimmune diseases
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toxicicty of azathiprine. What other drug could exacerbate its toxiciity?
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BM suppression; active metabolite is metabolized by Xanthine Oxidase so toxicity would be exacerbated with allopurinol
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what is OKT3?
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Muronomab-CD3 -> blocks T cell signal transduction
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clinical use of Muronab-CD3
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post kidney transplant
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toxicity of muronab-CD3
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hyperlipidemia, thrombocytopenia, leukopenia
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mechanism of Mycophenolate mofetil
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inhibits de novo guanine synthesis -> blocks lymphocyte production
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mechanism of Daclizumab
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IL-2 receptor on T cells -> downregulates proliferaton
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Adesleukin is a recombinant ____ and is used for
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IL-2; renal cell carcinoma, metastatic melanoma
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Erythropoiten is used for
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anemias, esp in renal failure
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Filgrastim is a recombinant ______ used for
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granulocyte colony stimulating factor; bone marrow recovery
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Sargramostim is a recombinant ______ used for
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granulocyte-MACROPHAGE-colony stimulating factor; used for bone marrow recovery
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alpha interferon is used therapeutically for
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Hep B, Hep C, Kaposi's sarcoma, leukemia, malignant melanoma
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beta interferon is used therapeutically for
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MS
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gamma interferon is used therapeutically for
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CGD
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Oprelvekin is a recombinant ______ used for
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IL-11; thrombocytopenia
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Thrombopoiten is used therapeutically for
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thrombocytopenia
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