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198 Cards in this Set

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