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198 Cards in this Set
- Front
- Back
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HLA DR5
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pernicious anemia & juvenile RA
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HLA DR2
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Goodpasture syndrome, allergy, multiple sclerosis, & narcolepsy
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HLA DR3
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celiac sprue, type I diabetes mellitus, & SLE
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HLA DR4
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pemphigus vulgaris, RA, type I diabetes
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HLA DR7
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steroid-responsive nephrotic syndrome
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HLA B27
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psoriasis, ankylosing spnondylitis, inflammatory bowel disease, Reiter's syndrome (PAIR)
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HLA B8
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Graves' disease, celiac sprue
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HLA DR5
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pernicious anemia (B12 deficiency), Hashimoto's thyroiditis
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antinuclear antibodies (ANA)
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SLE
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anti-dsDNA, anti-Smith
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specific for SLE
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antihistone
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drug-induced SLE
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anti-IgG (rheumatoid factor)
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rheumatoid arthritis
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antineutrophil (C-ANCA, P-ANCA)
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vasculitis
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anticentromere
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scleroderma (CREST)
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anti-Scl-70
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scleroderma (diffuse)
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antimitochondrial
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primary biliary cirrhosis
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antigliadin
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celiac disease
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anti-basment membrane
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Goodpasture's
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anti-epithelial cell
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pemphigus vulgaris
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antimicrosomal
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Hashimoto's thyroiditis
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anti-Jo-1
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polymyositis, dermatomyositis
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IL-1
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secreted by macrophages; stimulates T cells, B cells, neutrophils, fibroblasts, and epithelial cells to grow, differentiate or synthesize specific products
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this cytokine is an endogenous pyogen
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IL-1
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IL-2
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secreted by Th cells; stimulates growth of helper and cytotoxic T cells
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IL-3
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secreted by activated T cells; supports the growth and differentiation of bone marrow stem cells
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this cytokine has a function similar to GM-CSF
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IL-3
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IL-4
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secreted y Th cells; promotes growth of B cells; enhances cass switching of IgE and IgG
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IL-5
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secreted by Th cells; promotes differentiation of B cells; enhances class switching of IgA & stimulates production and activation of eosinophils
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IL-6
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secreted by Th cells and macrophages; stimiulates production of actute-phase reactants and immunoglobulins
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IL-8
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major chemotactic factor for neutrophils
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IL-10
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secreted by Th2 cells; stimulates Th2 while inihibiting Th1
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IL-12
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secreted by B cells and macrophages; activates NK and Th2 cells
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gamma-interferon
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secreted by Th cells; stimulates macrophages
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TNF-alpha
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secreted by macrophages; increases IL-2 receptor synthesis by Th cells; increases B cell proliferation; attracts and activates neutrophils; stimulates dendritic cell migration to lymph nodes
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what are the cell surface proteins on helper T cells?
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CD4, TCR, CD3, CD28, CD40L
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cell surface proteins on cytotoxic T cells
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CD8, TCR, CD3
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cell surface proteins on B cells
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IgM, B7, CD19, CD20, CD40, MHC II
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cell surface proteins on macrophages
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MHC II, CD14, receptors for Fc and C3b
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cell surface proteins on NK cells
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receptors for MHC I, CD 16
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cell surface protein on all cells except mature RBCs
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MHC I
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complement proteins involved in viral neutralization (4)
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C1, C2, C3, C4
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complement protein responsible for opsonization
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C3b
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complement proteins involved in anaphylaxis
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C3a, C5a
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complement protein responsbile for neutrophil chemotaxis
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C5a
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complement proteins involved in cytolysis by MAC
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C5b-9
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rheumatoid factor and anti-SS-A
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Sjogren syndrome
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HLA A3
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primary hemochromatosis
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high antibody titers to ribonucleoprotein (RNP); low titer RF and anti-ssDNA
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mixed connective tissue disease
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c-ANCA
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Wegener's granulomatosis
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anti-Ro/anti-SS-A
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Sjogren's syndrome
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anti-SS-B
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Sjogren's syndrome
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what instructs macrophages to become epitheliod and multinucleated giant cells?
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interferon-gamma
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what is the most specific marker for NK cells?
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CD56; also CD16
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cytokine that mediates the isotype switch to IgE in B lymphocytes
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IL-4, produced by Th2 cells
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what are the functions of lymph nodes?
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filtration by macrophages, storage/proliferation of B and T cells, antibody production
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what is the site of B cell localization and proliferation?
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follicle
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which follicles are dense and dormant?
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primary
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which follicles have pale central germinal centers and are active?
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secondary
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these communicate with efferent lymphatics and contain reticular cells and macrophages
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medulla
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what region of the lymph node houses T cells?
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paracortex
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in an extreme cellullar immune response, what part of the lymph node becomes greatly enlarged?
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paracortex
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this portion of the lymph node is not well developed in patients with DiGeorge syndrome
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paracortex
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what drains the right arm and the right half of the head?
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right lymphatic duct
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what drains everything but the right arm and right half of head?
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thoracic duct
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where are T cells found in the spleen?
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periarterial lymphatic sheath adn red pulp (T rex)
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where are B cells found within the spleen?
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white pulp
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these are long, vascular channels in red pulp with fenestrated "barrel hoop" basement membrane
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sinusoids of spleen
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what is found in the marginal zone of the spleen?
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APCs
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where do T cells mature? what is this derived from embryologically?
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thymus; epithelium of 3rd branchial pouches
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what part of the thymus is dense with immature T cells?
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cortex
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what part of the thymus is pale with mature T cells and epithelial reticular cells?
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medulla (M-mature/medulla)
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what part of the thymus contains Hassall's corpuscles?
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medulla
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MHC restriction occurs in what type of selection?
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positive
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nonreactive to self is associated with what type of selection?
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negative
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where do positive and negative selection occur in the thymus?
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corticomedullary junction
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these cells producse IL-2 and gamma-interferon
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Th1 cells
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these cells activate macrophages and Tc cells
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Th1 cells
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what do macrophages produces to influence naive helper T cells to differentiate into Th1 cells?
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IL-12
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what cells produce IL-4 and IL-5? what does this do?
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Th2 cells - causes B cells to differentiate into plasma cells
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what type of cells are responsible for host defense against infection with TB, virus-infected cells, and fungi?
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T cells
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what type of cells opsonize bacteria and neutralize toxins and viruses?
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B cells
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what type of cells are responsible for hay fever and Type I hypersensitivity reactions?
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B cells
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what type of cells are responsible for poison oak allergy and type IV hypersensitivity?
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T cells
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what type of cells are responsible for autoimmunity?
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B cells
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what type of cells are responsible for graft and tumor rejection and regulation of antibody response?
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T cells
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what MHC class consists of 1 polypeptide with beta2-microglobulin?
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MHC class I
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what MHC class consists of 2 polypeptides, and alpha and beta chain?
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MHC class II
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what cells have MHC I proteins?
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all nucleated cells
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what cells have MHC I and MHC II proteins?
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APCs
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what MHC class are the main determinants of organ rejection?
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MHC class II
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where does MHC I antigen loading occur?
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in RER (viral antigens)
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where does MHC II antigen loading occur?
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in acidified endosome
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helper T cells have ____, which binds to ____ on antigen-presenting cells
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CD4; MHC II
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cytotoxic T cells have ____, which binds to ____ on virus-infected cells
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CD8; MHC I
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this is a cluster of polypeptides associated with a T-cell receptor; it is important in signal transduction
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CD3 complex
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what does Th cell secrete that activates Tc cell to kill virus-infected cell?
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IL-2
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what do APCs express that bind to CD28 on Th cells to create the costimulatory signal?
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B7
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what part of an antibody recognizes antigens?
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variable part of L and H chains
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what part of antibody fixes complement?
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constant part of H chain of IgM and IgG
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what chain contributes to both Fc and Fab fractions?
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heavy chain
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what chain contributes only to Fab fraction?
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light chain
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how is antibody diversity generated?
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1. random recombination of VJ (light chain) or VDJ (heavy chain) genes 2. random combination of heavy chains with light chains 3. somatic hypermutation 4. addition of nucleotides to DNA during genetic recombination by tdt
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what mediates isotype switching?
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cytokines and CD40 ligand
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what is the main antibody in the secondary response?
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IgG
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what is the most abundant antibody?
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IgG
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this Ig type fixes complement, crosses the placenta, opsonizes bacteria, and neutralizes bacterial toxins and viruses
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IgG (Goes across placenta)
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this Ig type prevents attachment of bacteria and viruses to mucous membranes, does not fix complement
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IgA
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this Ig type is a monomer or dimer that is found in secretions and picks up secretory cmoponent from epithelial cells before secretion
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IgA
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this Ig type is produced in the primary response to an antigen
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IgM (priMary)
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this momomer or pentamer fixes complement but does not cross the placenta; antigen receptor found on the surface of B cells
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IgM
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this Ig type does not have a clear function; it is found on the surface of many B cells in serum
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IgD
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this Ig mediates immediate (type I) hypersensitivity by inducing the release of mediators from mast cells and basophils when exposed to allergen
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IgE
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this Ig has the lowest concentration in serum
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IgE
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this Ig mediates immunity to worms
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IgE
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this type of Ig epitope differs among members among members of same species; can be on light chain or heavy chain
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allotype (polymorphism)
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this is an Ig epitope common to a single class of Ig (5 classes, determined by heavy chainn)
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isotpe (IgG, IgA, etc.)
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this Ig epitope is determined by antigen-binding site; specific for a given antigen
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idiotype
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patients are given preformed antibodies after exposure to what four diseases?
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tetanus, botulinum, HBV, rabies (to be healed rapidly)
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this type of immunity is based on receiving preformed antibodies from another host
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passive
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this type of immunity is induced after exposure to foreign antigen - slow onset but long-lasting production
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active
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how does salmonella show antigen variation?
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two flagellar variants
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how does nenisseria gonorrhoeae show antigen variation?
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pilus protein
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what is anergy?
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self=reactive T cells become nonreactive without costimulatory molecule
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what types of hypersensitivity are antibody-mediated?
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I, II, III
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in this type of hypersensitivity, antigen cross=links IgE on presensitized mast cells and basophils, triggering release of vasoactive amines (e.g. histamine)
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type I (anaphylactic and atopic)
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anaphylaxis, asthma, hives, local wheal and flare are examples of what type of hypersensitivity?
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type I
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in this type of hypersensitivity, IgM, IgG bind to antigen on "enemy" cell, leading to lysis (by complement) or phagocytosis
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type II (cyotoxic)
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autoimmune hemolytic anemia, Rh disease, Goodpasture's, rheumatic fever, Grave's disease, bullous pemphigoid, MG, and ITP are all examples of what type of hypersensitivity?
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type II
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in this type of hypersensitivity, antigen-antibody complexes activate complement, which attracts neutrophils; neutrophils release lysosomal enzymes
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type III
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polyarteritis nodosa, immune complex glomerulonephritis, SLE, RA, serum sickness, and Arthus reaction are examples of what type of hypersensitivity?
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type III
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this is an imuune complex disease in which antibodies to foreign proteins are produced (takes 5 days); immune complexes are formed and deposit in membranes where they fix complement
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serum sickness (type III)
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this is a local subacute antibody-mediated hypersensitivity (type III) reaction in which intradermal injection of antigen induces antibodies which form Ag-Ab complexes in skin
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Arthus reaction
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this type III hypersensitivity reaction is characterized by edema, necrosis, and activation of complement
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Arthus reaction
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hypersensitivity pneumonitis (farmer's lung) and thermophilic actinomycetes are examples of what?
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Arthus reaction
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in this type of hypersensitivity, sensitized T lymphocytes encounter antigen and then release lymphokines, which leads to macrophage activation
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type IV (delayed/T-cell mediated)
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transplant rejections, TB skin tests, and contact dermatitis are examples of what type of hypersensitivity?
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type IV
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fever, urticaria, arthralgias, proteinuria, lymphadenopathy 5-10 days after drug exposure
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serum sickness
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what defends agains gram-negative bacteria?
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complement
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the classic pathway is activated by what?
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IgG or IgM (GM makes classic cars)
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what activates the alternate pathway?
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molecules on the surface of microbes (especially endotoxin)
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interferons induce the production of a 2nd protein that inhibits viral protein synthesis by doing what?
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degrading viral mRNA
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these are proteins that place uninfected cells in an antiviral state
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interferons (alpha, beta, gamma)
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which interferons inhibit viral protein synthesis?
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alpha and beta interferons
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what does gamma-interferon do?
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increases MHC I and II expression and antigen presentation in all cells
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this type of rejection is due to the presence of preformed antidonor antibodies in the transplant recipient
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hyperacute rejection
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this type of transplant rejection is cell-mediated due to cytotoxic T lymphocytes reacting against foreign MHCs
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acute rejection
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what type of transplant rejection is reverisble with immunosuppressants such as cyclosporin and OKT3?
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acute rejection
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this type of transplant rejection is characterized by antibody-mediated vascular damage (fibrinoid necrosis) and is irreversible
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chronic rejection
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what are major symptoms of graft-versus-host disease?
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maculopapular rash, jaundice, hepatosplenomegaly, and diarrhea
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what is the defect in Bruton's agammaglobulinemia?
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X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of Igs
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this disorder occurs in boys and is associated with recurrent bacterial infections after 6 months of age, when levels of maternal IgG antibody decline
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Brutons agammaglobulinemia (B cells)
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this immune deficiency presents with tetany owing to hypocalcemia and recurrent viral and fungal infections
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DiGeorge syndrome/thymic aplasia (T cells)
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this immune deficiency is associated with congenital defects of heart and great vessels
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DiGeorge syndrome
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what ist eh chromosomal abnormality associated with DiGeorge syndrome?
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22q11 deletion
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this is a defect in early stem cell differentiation and presents with recurrent viral, bacterial, fungal, and protozoal infections
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SCID - B and T cell deficiency
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failure to synthesize MHC II antigens, defective IL-2 receptors, and adenosine deaminase deficiency can all lead to what immune deficiency?
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SCID
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what does IL-12 receptor deficiency (decreased activation of T cells) present with?
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disseminated mycobacterial infections
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defect in CD40 ligand on CD4 T helper cells leads to inability to class switch; presents early in life with severe pyogenic infections
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hyper-IgM syndrome (decreased activation of B cells)
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this disorder is characterized by high levels of IgM and very low levels of IgG, IgA, and IgE
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hyper-IgM syndrome
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this syndrome is characterized by an X-linked defect in the ability to mount an IgM response to capsular polysaccharides of bacteria
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Wiskott-Aldrich syndrome (decreased activation of B cells)
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what is the triad of symptoms associated with Wiskott-Aldrich syndrome?
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infections, thrombocytopenic purpura, eczema (WIPE)
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this syndrome is associated with elevated IgA levels, normal IgE levels, and low IgM levels
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Wiskott-Aldrich syndrome
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this syndrome presents with recurrent 'cold' (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high IgE levels
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Job's syndrome (decreased activation of macrophages)
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this syndrome is characterized by failure of gamma-interferon production by helper T cells; neutrophils fail to respond to chemotactic stimuli
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JOb's syndrome
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what is the defect in leukocyte adhesion deficiency syndrome?
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defect in LFA-1 adhesion proteins on phagocytes
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this syndrome presents early with severe pyogenic and fungal infections and delayed separation of umbilicus
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leukocyte adhesion deficiency syndrome
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how is Chediak-Higashi inherited?
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autosomal recessive
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this disease is marked by a defect in microtubular function and lysosomal emptying of phagocytic cells
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Chediak-Higashi disease
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this disease presents with recurrent pyogenic infections by staph and strep, partial albinism, and peripheral neuropathy
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Chediak-Higashi disease
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defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes
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chronic granulomatous disease
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how is the diagnosis of chronic granulomatous disease confirmed?
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negative nitroblue tetrazolium dye reduction test
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this disease presents with marked susceptibility to opportunistic infections with bacteria, especially S. aureus, E. coli, and Aspergillus
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chronic granulomatous disease
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what is the defect in chronic mucocutaneous candidiasis present?
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idiopathic dysfunction of T cells specifically against candida albicans
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what is the most comon selective immunoglobulin deficiency? how does it present?
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selective IgA deficiency - presents with sinus and lung infections; milk allergies and diarrhea are common
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ataxia-telangiectasia is an idiopathic dysfunction of what type of cells?
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B cells
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defect in DNA repair enzymes with associated IgA deficiency; presents with cerebellar problems and spider angiomas
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ataxia-telangiectasia
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deficiency of what leads to hereditary angioedema?
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C1 esterase inhibitor
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deficiency of which complement protein leads to severe, recurrent, pyogenic sinus and RT infections?
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C3
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deficiency of what complement proteins leads to Neisseria bacteremia?
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C6-C8
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deficiency of what leads to paroxysmal nocturnal hemoglobinuria?
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decay-accelerating factor
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patients with what deficiency have an increased susceptibility to recurrent bacterial infections, especially with encapsulated bacteria
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C3 deficiency; not detected until later in life
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what is the most important immunological protective mechanism against blood-borne encapsulated organisms?
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IgG-mediated opsonization in the spleen
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Warthin-Finkeldy giant cell is pathognomonic for what?
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measles or the live attenuated measles vaccine
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Lyme arthritis is associated with what HLA?
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HLA-DR4
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patients with Wiskott-Aldrich syndrome have a 12% chance of developing what?
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non-Hodgkin's lymphoma
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test of choice to determine presence of circulating anti-Rh antibody?
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indirect Coombs test to measure IgG anti-Rh antibody
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low levels of all antibody classes
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common variable immunodeficiency
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spleen is important for removing what type of organisms? list 3
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strep pneumo, H. flu, Neisseria
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what is defective in leukocyte adhesion deficiency?
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integrins - function both in adhesion of leukocytes to other cells and in the phagocytosis of complement-coated material
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HLA types associated with SLE?
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HLA-DR2 and HLA-DR3
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most likely sequelae of rheumatic fever?
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mitral valve disease
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antitopoisomerase antibodies?
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scleroderma/systemic fibrosis - likely to develop diffuse systemic fibrosis & death from consequences of systemic disease such as pulmonary fibrosis or malignant hypertension
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what is responsible for strong binding between monocytes, T lymphocytes, macrophages, neutrophils, and dendritic cells, and injured epithelium?
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LFA-1; interacts with ICAM-1
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an increased level of what cytokine would decrease the likelihood of a delayed-type hypersensitivity reaction?
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IL-10
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what 2 molecules exert the most powerful chemotactic effect on neutrophils?
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C5a and C8
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what are the best markers for identification of B cells?
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CD19, CD20, CD21
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IgG subclass deficiency is associated with a deficiency with what other substrate?
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IgA
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what is C-reactive protein a marker of?
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non-specific inflammation - one of the most commonly measured acute-phase reactants
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