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129 Cards in this Set
- Front
- Back
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graves disease
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hyperthyroidism
anti-TSH receptor antibodies stimulate production of thyroid hormones treatment - thyroid gland ablation to stop production of hormones |
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Hashimoto's Thyroiditis
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hypothyroidism
lymphocytes enter thyroid gland, produce antibodies against thyroid hormones, destroy normal thyroid tissue treatment - replacement therapy |
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what is rheumatoid factor?
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IgM anti-IgG antibodies
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what is the most common rheumatic disease?
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rheumatoid arthritis
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rheumatoid arthritis
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immune complex disease
infiltration of joints by leukocytes inflammatory response in joints exacerbated by rheumatoid factor (IgM anti-IgG antibodies) treatment - anti-inflammatory and immunosuppressive drugs (anti-TNFalpha therapy) |
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systemic lupus erythematosis
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circulating auto-antibodies against cell surface
anti-DNA/RNA antibodies anti-nucleoprotein antibodies deposition of complexes in tissues results in more inflammation characteristic butterfly rash caused by complexes in the skin |
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what is epitope spreading?
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development of immune responses to epitopes distinct from, and noncross-reactive with, the dominant part of an antigen that is recognized by the immune system, specifically by antibodies
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multiple sclerosis
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T-cell mediated disease against myelin basic protein and proteolipid protein
antibodies against myelin, causing loss of myelin sheath treated with beta-interferon |
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Guillain-Barre Syndrome
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acute inflammatory polyneuropathy
immune reaction against peripheral nerves, caused by viral or campylobactor jejuni infection ascending peripheral muscle weakness leading to paralysis type II and type IV hypersensitivities |
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myasthenia gravis
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auto-antibodies against acetylcholine receptor, causing loss of receptors on muscle cells
progressive muscle weakness, often first seen in facial muscles treated with pyridostigmine to inhibit cholinesterase |
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what is diplopia?
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double vision
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what is pyridostigmine?
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chonlinesterase inhibitor
(increases half life of ACh) used to treat myasthenia gravis |
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celiac disease
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immune response to wheat gluten (gliadin) resulting in IgG/IgA against transglutaminase
villous atrophy and malabsorption caused by immune response treated by avoiding gluten-containing foods |
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autoimmune hemolytic anemia
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type - II (antibody against cell-surface or matrix antigens)
autoantigen - Rh blood group antigens, I antigen destruction of red blood cells by complement and phagocytes (anemia) |
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autoimmune thrombocytopenia purpura
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type - II (antibody against cell-surface or matrix antigens)
autoantigen - platelet integrin gpIIb:IIIa abnormal bleeding (loss of clotting) |
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Goodpasture's Syndrome
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type - II (antibody against cell-surface or matrix antigens)
autoantigen - non-collagenous domain of basement membrane, and collagen type IV consequence - glomerulonephritis, pulmonary hemorrhage |
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Pemphigus Vulgaris
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type - II (antibody against cell-surface or matrix antigens)
autoantigen - epidermal cadherin consequence - blistering of skin |
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acute rheumatic fever
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type - II (antibody against cell-surface or matrix antigens)
autoantigen - cross reaction of antibodies against stretococcal cell wall which cross react with cardiac muscle consequence - arthritis, myocarditis, late scarring of heart valves |
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subacute bacterial endocarditis
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type - III (immune-complex disease)
autoantigen - bacterial antigen consequence - glomerulonephritis |
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mixed essential cryoglobulinemia
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type - III (immune-complex disease)
autoantigen - rheumatoid factor IgG complexes (with or without hepC antigens) consequence - systemic vasculitis |
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Systemic Lupus Erythematosus
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type - III (Immune-complex disease)
autoantigen - DNA, histones, ribosomes, snRNP, scRNP consequence - glomerulonephritis, vasculitis, arthritis |
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Type 1 diabetes (insulin-dependent diabetes mellitus)
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type - IV (Tcell-mediated disease)
autoantigen - pancreatic beta cell antigen consequence - beta cell destruction |
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rheumatoid arthritis
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type - IV (Tcell-mediated disease)
autoantigen - unknown synovial joint antigen consequence - joint inflammation and destruction |
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multiple sclerosis
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type - IV (Tcell-mediated disease)
autoantigen - myelin basic protein, proteolipid protein consequence - brain degeneration, paralysis |
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when were immuodeficiencies first identified?
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1950's
(with advent of antibiotics) |
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if a child is having recurrent bacterial infections, what are his/her potential immunodeficiencies?
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complement not working
phagocytes not working no antibodies/B cells not working |
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if a child is having recurrent viral or fungal infections, what are his/her potential immunodeficiencies?
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T cells not working
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if a child is infected with rare or generally benign pathogens, what are his/her potential immunodeficiencies?
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no immune system
no Th cells |
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what is another name for Bruton's disease?
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X-linked agammaglobulinemia (XLA)
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what was the first immunodeficiency described?
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Bruton's disease in 1952
(defective BTK gene) |
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what causes X-linked agammaglobulinemia?
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aka Bruton's disease
defective gene for Bruton's Tyrosine Kinase (important for preB cell signaling) |
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with what is Bruton's disease treated?
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gamma globulin injections
(passive immunity) |
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absence of which immunoglobulins suggests an early B cell defect?
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IgA
IgM IgG |
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what is the most common immunodeficiency?
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Selective IgA deficiency
(1 in 800 births) |
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selective IgA deficiency
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genetic basis unknown
decreased release of IgA recurrent sinopulmonary infections (viral and bacterial) treat with antibiotics |
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what is another name for common variable immunodeficiency?
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acquired hypogammaglobulinemia
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common variable immunodeficiency
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cause is unclear
wide range of B cell defects highest frequency 15-35 years old recurrent infections with pyogenic bacteria increased incidence of autoimmune disease (SLE, thrombocytopenia) treat with immune serum globulin |
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DiGeorge's Syndrome
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Defect in embryonic development of 3rd and 4th pharyngeal pouches
thymic aplasia -> recurrent viral infections hypoparathyroidism heart defects treat with fetal thymus transplant |
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chronic granulomatous disease (CGD)
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defect in NADPH oxidase, so reactive oxygen species can't be produced
chronic bacterial infection granulomas form in patient (phagocytes filled with ingested bacteria) symptoms in first two years diagnose with nitroblue tetrazolium dye |
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Leukocyte Adhesion Deficiency (LAD)
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defect in CD18 (common subunit of several integrins like LFA1, CR2, CR4), so phagocytes can't migrate to site of infection
chronic bacterial infections |
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glucose-6-phosphate dehydrogenase deficiency
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deficiency of G6PDH
defective respiratory burst impaired killing of phagocytosed bacteria chronic bacterial and fungal infections anemia induced by certain agents |
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myeloperoxidase deficiency
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deficiency of myeloperoxidase in neutrophil granules and macrophage lysosomes
impaired production of toxic oxygen species impaired killing of phagocytosed bacteria chronic bacterial and fungal infections |
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Chediak-Higashi syndrome
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defect in vesicle fusion
impaired phagocytosis due to inability of endosomes to fuse with lysosomes recurrent and persistent bacterial infections granulomas |
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what are the effects of a deficiency in C1, C2, or C4?
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immune complex disease
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what are the effects of a deficiency of C3?
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susceptibility to capsulated bacteria
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what are the effects of a deficiency of C5, C6, C7, C8, C9?
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susceptibility to Neisseria
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what are the effects of a deficiency of Factor D or Factor P (properdin)?
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susceptibility to capsulated bacteria and Neisseria, but no immune complex disease
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what are the effects of a deficiency of Factor I?
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susceptibility to capsulated bacteria
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what are the effects of DAF or CD59?
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autoimmune-like conditions
(paroxysmal nocturnal hemoglobinuria) |
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what are the effects of a deficiency of C1INH?
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hereditary angioneurotic edema (HANE)
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Hereditary Angioneurotic Edema (HANE)
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aka Hereditary Angioedema
defect in C1 inhibitor (C1INH) uncontrolled C1 activation of classical pathway increase in vasoactive components (C2a) local edema in organs (airway obstruction and death by suffocation) |
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what is SCID?
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severe combined immunodeficiency
no Tcell dependent antibody responses no cell-mediated immune responses |
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what is BLS1?
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bare lymphocyte syndrome 1
defect in Tap gene no MHC class 1 expression no CD8 Tcells pulmonary and viral infections |
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which type of bare lymphocyte syndrome is the less severe?
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BLS1 is less severe than BLS2
BLS1 - defect in Tap gene BLS2 - defect in CIITA or RFX |
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what is BLS2?
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bare lymphocyte syndrome 2
defect in CIITA or RFX gene, which is required for MHC class II expression no MHC class II no T helper cells |
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what is the most common form of SCID?
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X-linked SCID
mutation in common gammaC chain |
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X-linked SCID
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mutation in common gammaC chain
loss of functional cytokine receptors (IL2, IL4, IL7, IL9, IL15) failure to interact with JAK3 kinase failure in Tcell and NK cell development B cells do not proliferate in response to IL4 |
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how is a patient treated who has X-linked SCID?
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isolated in pathogen-free environment
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what is ADA?
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adenosine deaminase
key enzyme in purine metabolism |
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ADA deficiency
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causes SCID (autosomal recessive inheritance)
nucleotide metabolites (ATP, dATP) accumulate in all cells, but are especially toxic to T cells |
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what is PNP?
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purine nucleoside phosphorylase
enzyme in purine catabolism |
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PNP deficiency
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causes SCID
buildup of dGTP, which is toxic to T cells |
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Omenn's Syndrome
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defect in RAG gene
no antigen receptors on B or T cells |
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Wiskott-Aldrich Syndrome
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x-linked gene
cytoskeletal reorganization --thrombocytopenia --infections treated with bone marrow transplant |
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how is ADA deficiency treated?
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bone marrow transplant or gene therapy
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what is AIDS?
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acquired immunodeficiency syndrome
slowly progressive diseased, caused by HIV virus infecting and killing CD4 cells treatment - antivirals |
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how does HIV enter CD4 cells?
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CCR5
CXCR4 |
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what factors can cause secondary immunodeficiencies?
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poor nutrition
chemotherapy immunosuppressive drugs autoimmune disease loss of lymphoid organs chronic infections |
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when was the first blood transfusion?
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1812
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isohemagglutinins
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agents that cause agglutination of red blood cells
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what is the result of the wrong blood type in transfusion?
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hemolytic anemia
type II hypersensitivity |
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what blood type is the universal donor?
universal recipient? |
universal donor = type O (O-positive)
universal recipient = type AB (AB-negative) |
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erythroblastosis fetalis
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Rh-negative mother gives birth to an Rh-positive child, developing antibodies to the child's blood cells
during subsequent pregnancies, mother's anti-Rh IgG antibodies cross placenta and destroy fetal red blood cells |
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how is erythroblastosis fetalis treated/avoided?
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Rh negative mother is treated with Rhogam, which is an anti-Rh serum
binds to and destroys Rh+ fetal red blood cells so that Rh- mother does not become sensitized to them (creating IgG that will cross placenta) |
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what are the types of organ transplants?
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autograft - from yourself (e.g. skin)
isograft (syngraft) - from genetically identical person allograft - from genetically dissimilar person xenograft - from different species |
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what is HLA?
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human leukocyte antigens
aka major histocompatibility complex (MHC) alloantigens which are highly polymorphic and vary between people generate immune response against graft |
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how are MHC genes inherited?
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genetic recombination is suppressed, so children receive same set of alleles as parents
both parents have two sets of six different HLA alleles |
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what are the types of organ rejections?
how long do they take? |
hyperacute - minutes to hours - pre-formed antibodies in recipient
acute - days to weeks - cell mediated immunity in recipient chronic - months to years - cell mediated and antibody immunity in recipient |
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hyperacute allograft rejection
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minutes to hours after transplant
caused by pre-formed antibodies in recipient (to ABO or HLA antigens on vascular endothelium of transplanted tissue) activate complement cannot be stopped |
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acute allograft rejection
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days to weeks after allograft transplant
cell-mediated immunity in recipient leukocytes infiltrate graft and activate CMI and inflammatory response caused by HLA mismatch |
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with what is acute allograft rejection treated?
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treated with immunosuppressive drugs
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what are the pathways involved with both acute and chronic allograft rejections?
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direct - donor DC present antigen to recipient T cells
indirect - recipient DC take up donor MHC from donor DC and present to recipient T cells |
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chronic allograft rejection
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months to years after allograft transplant
cell-mediated and antibody immunity in recipient inflammatory response in blood vessels, caused by leukocyte infiltration of graft; leads to fibrosis of graft graft cannot be saved |
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direct pathway of allograft rejection
(acute and chronic rejection) |
donor dendrite cells enter recipient lymph nodes and present antigens to recipient T cells (activating them)
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indirect pathway of allograft rejection
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dendrite cells from donor organ die and their MHC is taken up by the recipient dendritic cells, which then present the antigenic donor MHC peptides to recipient T cells (activating them)
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corticosteroids
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ex. prednisone
used for anti-inflammatory properties affect gene expression in lymphocytes side effects - fluid retention, weight gain, diabetes |
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prednisone
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type of corticosteroid
converted to active form (prednisolone) in patient |
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cytotoxic immunosuppressive drugs
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ex. azithroprine, cyclophosphamide, methotrexate
block DNA replication and cell division |
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azithroprine
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type of cytotoxic immunosuppressive drug
inhibits DNA replication in all dividing cells |
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cyclophosphamide
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type of cytotoxic immunosuppressive drug
alkylates and crosslinks DNA nitrogen mustard from WWI |
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methotrexate
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type of cytotoxic immunosuppresive drug
inhibits dihydrofolate reductase used for bone marrow transplant recipients to block GVHD |
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T cell inhibitor class of immunosuppressive drugs
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cyclosporin A
FK506 (tacrolimus) Rapamycin (sirolimus) |
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cyclosporin A
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T cell inhibitor class of immunosuppressive drugs
binds cyclophilin, forming a complex which binds calcineurin, preventing activation of NFAT; without NFAT activation (binding to AP-1) transcription is lost |
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FK506
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T cell inhibitor class of immunosuppressive drugs
binds FK-binding protein (FKBP), forming a complex which binds calcineurin, preventing activation of NFAT; without NFAT activation (binding to AP-1) transcription is lost |
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calcineurin
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phosphatase that is activated by raised intracellular calcium concentration and then activates NFAT, which forms an active transcription factor with AP-1
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what are the effects of cyclosporin A and tacrolimus on T lymphocytes?
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reduced IL2, IL3, IL4, GM-CSF, and TNFalpha
reduced cell division because of decreased IL2 reduced calcium dependent exocytosis of cytotoxic granules inhibition of antigen driven apoptosis |
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what are the effects of cyclosporin A and tacrolimus on B lymphocytes?
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inhibition of cell division because T-cell cytokines are absent
inhibition of antigen-driven cell division induction of apoptosis after B-cell activation |
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what are the effects of cyclosporin A and tacrolimus on granulocytes?
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reduced calcium-dependent exocytosis of granules
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what antibodies are used against T cells?
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mouse monoclonal antibodies
anti-CD4 anti-CD3 anti-CD25 |
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bone marrow transplantation
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replace entire hematopoietic system
try to fix defect in patients with genetic diseases replaces blood system in patients undergoing high dose chemotherapy/radiaion therapy for cancer |
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graft versus host disease (GVHD)
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T cells in graft attack recipient
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autologous transplant
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bone marrow transplantation in which hematopoietic stem cells from patient are isolated, removed, and used for transplant
fewer problems |
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what genetic changes in a cell can cause cancer?
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loss of tumor suppressor gene
mutation of oncogene mutation of DNA repair gene loss of cell cycle control |
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papillomavirus
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DNA virus associated with:
warts (benign) carcinoma of uterine cervix worldwide |
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hepatits B virus
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DNA virus associated with:
liver cancer (hepatocellular carcinoma) prevalent in southeast asia and tropical africa |
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epstein-barr virus
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DNA virus associated with:
burkitt's lymphoma (cancer of B lymphocytes) nasopharyngeal carcinoma B-cell lymphoproliferative disease |
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human T-cell leukemia virus type 1 (HTLV-1)
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RNA virus associated with adult T-cell leukemia/lymphoma
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human immunodeficiency virus (HIV-1)
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RNA virus associated with kaposi's sarcoma
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human herpes virus 8 (HHV8)
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RNA virus associated with kaposi's sarcoma
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why are cancer cells not seen initially by the immune system?
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no inflammatory response
self MHC (once they are seen, the tumor load may overwhelm the immune system) |
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how are cancers recognized by the immune system?
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mutations in cancer cells express new genes, often embryonic genes
MHC gene expression is down-regulated |
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how do tumor cells evade the immune system?
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new genes may not be presented well by MHC
tumor cells down regulate MHC, possibly to the point of losing class I or II tumor cells secrete TGFbeta to keep immune system from making a strong response no inflammatory response during presentation (DCs without B7 present tumor antigens become anergized) |
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Coley's Toxin
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developed by NY surgeno William Coley in early 1900's
infected cancer patients with bacteria associated sarcoma regression with bacterial infection, believed to utilize TNF |
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what are the four types of immunotherapies for cancer treatment?
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cytokine
antibody adoptive vaccine |
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what are the types of antibody cancer immunotherapies?
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ADCC - activates macrophages
Complement - activates complement toxins - attached toxins activated after entry to tumor cells radionuclide - radiation from antibodies kills surrounding cells |
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rituximab
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monoclonal antibody cancer treatment
antigen - CD20 (B cell signaling receptor) treats Non-Hodgkin's lymphoma |
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trastuzumab
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monoclonal antibody cancer treatment
antigen - HER2/neu protein (growth factor receptor) treats breast cancer |
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alemtuzumab
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monoclonal antibody cancer treatment
antigen - CD52 (differentiation antigen) treats chronic lymphocytic leukemia |
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cetruximab
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monoclonal antibody cancer treatment
antigen - epidermal growth factor receptor (growth factor receptor) treats colorectal cancer and head and neck cancer |
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panitumumab
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monoclonal antibody cancer treatment
antigen - epidermal growth factor receptor (growth factor receptor) treats colorectal cancer |
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bevacizumab
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monoclonal antibody cancer treatment
antigen - vascular endothelial growth factor (promotes angiogenesis) treats colorectal cancer and non-small cell lung cancer |
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gemtuzumab
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conjugated monoclonal antibody cancer treatment
antigen - CD33 conjugate - ozogamicin (cytotoxic antibiotic derivative) treats acute myelogenous leukemia |
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ibritumomab
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conjugated monoclonal antibody cancer treatment
antigen - CD20 conjugate - indium-111 for imaging, yttrium-90 for treatment, via tiuexetan (chelator) treats Non-Hodgkin's lymphoma |
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tositumomab
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conjugated monoclonal antibody cancer treatment
antigen - CD20 conjugate - iodine-131 treats Non-Hodgkin's lymphoma |
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how do antibody-toxin conjugates kill tumor cells?
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antibody-toxin conjugate recognizes antigen on tumor cell and is internalized
toxin is cleaved from antibody, activated and passes to nucleus toxin induces double-strand breaks in nuclear DNA |
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how do antibody-radionuclide conjugates kill tumor cells?
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radioactive antibody binds to antigen on tumor cells and irradiates them
radiation damages the cells' DNA and kills them |
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what are the types of cytokine therapy used for cancer treatment?
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TNFalpha (severe side effects)
IL2 (severe toxicity) --renal carcinoma, melanoma (low response rate) IFNalpha (activates immune system) --kidney cancer (5-10% response) --leukemias and melanomas |
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what are the types of adoptive cancer therapy?
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dendritic cells - pulsed with tumor antigens and returned to patient
tumor infiltrating lymphocytes - isolated from tumor, expanded and returned to patient |
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what types of vaccines are used as tumor therapies?
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peptides - tumor specific proteins
mixtures - tumor proteins and/or tumor lysates modified tumor cells - express immune stimulating genes modified viruses - target tumor and bring toxic gene |
