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115 Cards in this Set
- Front
- Back
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What type of T-Cell (and subset) is responsible for Type I hypersensitivity reactions?
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1. CD4 TH2 cells
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What IL is responsible for the production of IgE?
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IL-4
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What cytokines are important in the involvement of eosinophils in Type I hypersensitivity?
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1. IL-3
2. IL-5 3. GM-CSF |
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What are the two phases of a Type I hypersensitivity reaction and what participates in each?
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1. Initial phase - 30 min - histamine, proteases, chemotactic factors and glycoproteins
2. Delayed phase - 2 to 24h - lipid mediators ie: LTB, LTC, LTD, LTE, PGD4, PDGF |
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What are the proinflammatory cytokines releases in a Type I hypersensitivity reaction?
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1. TNF
2. IL-1,3,4,5,6 3. GM-CSF 4. Chemokines (eotaxin, rantese) |
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What is another name for Local immediate hypersensitivity reactions?
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Atopic allergies - genetic predisposition
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Name the three pathological pathways in Type II hypersensitivity and describe how they work.
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1. Opsonization/Complement/Fc mediated phagocytosis - basically complement makes the MAC, antibody or C3b causes opsonization and NK cells with Fc receptors attack
2. Complement/Fc - binding of complement and antibodies cause recruitment of non-specific imflammatory cells that release ROIs and such 3. Antibodies that don't cause tissue damage cause cellular dysfunction |
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What is the target antigen in autoimmune thrombocytopenic purpura?
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Platelet membrane proteins (GpIIb:IIIa)
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What is the target antigen in pemphigus vulgaris?
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Cadherins
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What is the target antigen in Goodpasture's syndrome?
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Non-collagenous basement membrane protein that is in kindey glomeruli and lung alveoli
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What is the target antigen in pernicious anemia?
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Intrinsic factor of the gastric parietal cells.
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What do immune complexes cause to aggregate and what can they activate? What does this lead to?
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1. Platelets
2. Factor XII 3. Activation of the coagulation cascade and thrombis formation |
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What histological changes are seen in chronic serum sickness?
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Die to the chronic activation of inflammatory responses, one begins to see intimal fibrosis and parenchymal scarring.
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What are two pathologic processes involved in Type IV hypersensitivity?
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1. CD4 and CD8 cytokine release that causes inflammation and tissue damage
2. CD8 mediated cytotoxicity |
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In delayed type IV hypersensitivity, what IL actiavtes which type of T-Cell? What happens after that?
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Macrophages release IL-12 which activates CD4 TH1 cells that release INF-G(potent macrophage activator), IL-2(acivates T Cells) and TNF(proinflammatory).
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What are the two ways that host T Cells recognize allograft HLA molecules?
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1. Direct pathway - CD8 cells bind to the transplant APC MHCI and recognize it as forgein and begin attacking. CD4 recognize the MHC II as foreign and turn into effector CD4 cells (make antibodies and cytokines)
2. Indirect - CD4 cells are presented foreign material after is has been processed and presented by host APCs. This is like a normal immune response. |
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What are the two types of actue rejection?
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1. Acute cellular rejection - cell-mediated
2. Acute humoral rejections - antibody mediated |
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What is the main histological change in chronic rejection?
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Obliterative initmal fibrosis in arteries of the donor tissue.
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What cells are responsible for rejection of hematopoietic cells?
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NK and radiation resistant T-cells
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What is a big complication in some bone marrow transplant cases? Why does it happen?
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1. Graft vs Host disease
2. This occurs because the donor tissue will still have live T Cells within it. These T Cells recognize the non-identical HLA molecules on the recipient T Cells and foreign. They then begin an immune response and attack. |
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Why aren't the T Cells in bone marrow transplants killed before they are implanted into the host?
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T Cell deficient bone marrow transplants lead to a high rate of leukemia. There seems to be a big Graft vs. Leukemia reaction happening in transplants that protects the recipient from leukemia.
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Name four different mechanisms for the inactivation of self-reactive T Cells.
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1. Anergy - occurs when a self reactive T Cell does not get the co-stimulatory signals it needs.
2. Suppression by regulatory T Cells - regulatory T cells release IL-10 and TGF-B which are inhibitory 3. Clonal deletion - occurs when there is persistant activation of cells by a self-antigen. It occurs by the up-regulation of Fas-L which induces apoptosis 4. Sequestering of antigens - immunopriveledged sites |
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How could infections cause induce an autoimmune response?
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1. Infections can upregulate co-stimulatory molecules needed to overcome peripherial tolerance
2. Molecular mimicry 3. Damage to normal tissue caused by the infection might damage previously tolerated antigens causing the immune system to recognize them as forgein. 4. Damage due to the infection could liberate previously cryptic self-antigens that the immune system has not seen before. |
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What phenomenon causes autoimmune disorders to be persistent?
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Epitope switching
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What two anti-bodies are highly suggestive (almost diagnostic) of SLE?
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1. Anti-dsDNA
2. Anti-Sm (RNP) |
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40% to 50% of SLE patients have antibodies to what?
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They have antiphospholipid antibodies which are actually antibodies to a protein (Carbolipin) which when bound to membrane phospholipids exposes the antibody binding site.
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Lupus anticoagulants cause what phenomenon?
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They actually cause hypercoagability in vivo.
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According to current research, what is the problem with people who have SLE?
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They seem to have lost peripherial B Cell tolerance. This leads to the production of autoantibodies that can cause Type II and Type III hypersensitivity reactions.
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What is typical in all tissues in SLE?
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An acute Type III reaction with acute necrotizing vasculitis and fibrinoid deposits involving small arteries and arterioles.
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What are the five classes of lupus nephritis?
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1. Class I
2. Class II - mesangial lupus glomerulonephritis 3. Class III - focal proliferative GN 4. Class IV - diffuse prolifertive GN (sub-endothelial complex deposition and wire-loops) 5. Class V - Membranous GN (sub-epithelial complex deposition) |
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What are some of the histological changes seen in the skin of people with SLE(4)?
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1. Deposition of complement and immunoglobins at the dermal-epidermal junction
2. Fibrosis 3. Perivascular monocyte infiltration 4. Vascular fibrinoid change |
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What are characteristics of the SLE affected joint?
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1. Non-specific, nonerosive synovitis
2. Minimal joint deformity |
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How does SLE affect the heart?
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It mainly causes pericarditis. It may also cause non-bacterial verrucous (Libman-Sacks) endocarditis which may lead to stenosis or regurgitation.
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What affect does SLE have on the spleen?
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1. Capsular thickening and follicular hyperplasia
2. Splenomegaly 3. Onion-skin appearance |
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What affect does SLE have on the lungs?
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Pleuritis w/ pleural effusions in 50% of SLE patients.
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What is the ten year survival rate of SLE patients?
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80%
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What is chronic discoid lupus erythematosus? What percentage of people have ANA? How is it similar to SLE? Over time, what happens to 5% to 10% of the patients?
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1. It is a SLE like disease that is limited to the skin.
2. 30% - anti-histone 3. They both have complement and immunoglobin deposition at the dermis-epidermis layer 4. Becomes systemic |
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What drugs can cause drug-induced lupus erythematosus?
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1. Procainamide
2. Hydralazine 3. Isoniazid 4. D-penicillamine |
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In drug induced LE, what type of ANA is mainly seen?
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Anti-histone antibodies
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There is linkage with what in drug induced LE?
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HLA-DR4
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What two things characterize Sjogren syndrome? Who does it mainly affect?
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1. Dry eyes (keratoconjunctivitis sicca)
2. Dry mouth (xerostomia) Both are due to the destruction of the lacrimal and salivary glands 3. 90% women ages 35-45 |
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What ANA is prototypical of Sjogrean syndrome?
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1. Anti SS-A
2. Anti SS-B Both are ribonucleotide proteins |
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What other diseases has there been linkage to in regards to Sjogren syndrome?
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1. Epstein-Barr
2. Hepatitis C 3. HTLV-1 4. HIV |
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What are three things seen histologically in Sjogren syndrome?
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1. Periductal CD4 T Cell invasion
2. Ductal hyperplasia 3. Luminal obstruction |
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What extraglandular involvement can be caused by Sjogren Syndrome?
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1. Tubulointerstitial nephritis with tubular atrophy
2. Adenopathy (40x increased risk of B cell lymphoma) |
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What is Raynaud phenomenon? What condition is it associated with?
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1. It is paroxysmal pallor or cyanosis of the tips of the fingers and toes.
2. Systemic sclerosis |
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What causes dysphagia in 50% of systemic sclerosis patients?
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Fibrosis of the esophagus
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What are the two subsets of systemic sclerosis?
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1. Diffuse sclerosis - rapid progression
2. Localized (CREST) - late visceral involvement with a typically benign course |
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What ANA is typically seen in diffuse systemic sclerosis?
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Anti-DNA topoisomerase I Antibody
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What does CREST stand for?
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1. Calcinosis
2. Raynaud phenomenon 3. Esophageal dysmotility 4. Sclerodactyly 5. Telangiectasia |
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What is the pathogenesis involved with systemic sclerosis?
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Basically it starts with vascular injury due to autoimmune response. This leads to the productions of growth factors/cytokines (IL1 IL4 PDGF TNF TGF) that promotes collagen synthesis.
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What ANA is typically involved with CREST syndrome?
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Anti-centromere antibody
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What accounts for 50% of the deaths in systemic sclerosis?
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Renal failure marked by increased luminal fibrosis and hypertension.
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What problems are seen in the heart with systemic sclerosis (2)?
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1. Cardiac myopathy
2. Conduction problems leading to arrhythmias. |
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What is mixed connective tissue disease? What ANAs are not seen in it?
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1. It is a hodge-podge of autoimmune diseases.
2. Does not have Anti-dsDNA or anti-Sm like SLE |
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What is the defect in X-Linked Brutons Agammaglobulinemia?
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There is a severe lack of mature B Cells due to a defect in Bruton's Tyrosine Kinase (Btk). Without it light-chains are not produced so immature B Cells never mature.
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People with X-Linked Brutons Agammaglobulinemia also have an increased risk of getting what?
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Connective tissue auto-immune disorders
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In isolated IgA deficiency, what other immunoglobins might you be defiecient in?
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1. IgG2
2. IgG4 |
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What is the defect in IgA deficiency?
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Failure of maturation of immature IgA producing B Cells.
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What is a precaution that needs to be taken with someone who has IgA deficiency?
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40% of these people have IgA antibodies and can have an anaphylactic reaction to blood transfusions containing IgA.
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In hyper-IgM syndrome, what is the main MOI?
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X-linked
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What is the defect in X-linked hyper-IgM syndrome?
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T Cells with defective CD40L needed to activate isotype switching in B Cells.
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What are two possible causes of hyper-IgM syndrome (non X-linked).
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Defective enzyme - Activation Induced Deaminase
or Defective CD40R on B Cells |
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What bacteria are people with hyper-IgM syndrome usually infected with?
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Pneumocystis jiroveci
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In X-linked SCID, where is the mutation?
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In one of the common Gamma chains in several cytokine receptors.
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Which IL is needed for the proliferation of lymphocyte progenitor cells?
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IL-7
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In autosomal recessive SCID, where is the defect?
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In the enzyme adenosine deaminase.
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What marks Wiscott-Aldrich syndrome(4)?
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1. Thrombocytopenia
2. Eczema 3. Predilication for lymphomas 4. Low peripheral T Cell counts |
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What is the defect in WAS?
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There is a defect in the production of WAS protein which is used to link surface receptors to cytoskeletal elements. This causes a reduction in cell signalling
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A deficiency of which complement factor is most common? Which is causes the most infection? Which are important in immune complex clearance?
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1. C2
2. C3 3. C1q and C4 |
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A defect in what enzyme of the complement system causes chronic angioedema?
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C1 esterase inhibitor
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What are the two membrane glycoproteins associated with HIV? What is the major capsid protein in HIV?
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1. GP120
2. GP41 3. p24 |
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What receptor does gp120 bind to in order to infect a T Cell? What are the co-receptors for infection?
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1. CD4
2. Chemokine receptors CCR5 and CXCR4 |
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What is an alternative way that CD4 T Cells can be destroyed in HIV?
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1. The destruction of the architecture of lymphoid organs causes including cells needed to support CD4 T Cells.
2. Chronic activation of unaffected cells 3. Fusion of cells 4. gp120 proteins sticking to cells marking them to death by CD8 cells |
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What is the main pathogenic process in CNS involvement seen in HIV?
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Circulating infected monocytes migrate to the brain where they can release cell damaging cytotoxic substances or recruit neuron damaging cells.
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What is an important predictor in the progression of an HIV patient?
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Their viral load once their T Cell count returns to normal in the actue phase of infection.
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What is the most common malignant neoplasm seen in HIV patients that have progressed to AIDS?
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Kaposi sarcoma (most likely infected with KS herpesvirus first)
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Cells infected with KS herpesvirus have a tendancy to progress to what?
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B Cell lymphoma
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What type of lymphoma is 120x more common in AIDS patients?
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Agressive B-Cell Non-Hodgkins Lymphoma
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Amyloid light chain proteins are usually associated with what diseases? What light chains type are they usually made of?
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1. B Cell dyscarias (Multiple Myelomas)
2. Lambda |
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Amyloid associated amyloidosis is made up of what protein made where?
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1. Serum Amyloid A
2. Liver |
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AB2 amyloid bodies are seen in what disease? What is the precursor for the amyloid bodies?
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1. Alzheimer Disease
2. APP |
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What is the normal protein that transports retinol and thyroxine that is responsible for familial amyloid polyneuropathy?
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Transthyretin
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Secondary or reactive amyloidosis uses what type of protein?
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AA bodies
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Hemodialysis related amyloidosis involves which protein?
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B2 microglobin
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Nodular amyloidosis involves which protein?
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AL
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In Sago spleen, deposits are limited to what areas?
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Splenic follicles
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In lardaceous spleen, deposits are limited to what areas?
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Splenic sinusoids
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What particular T Cell is the cause of the problems seen in Sjogren syndrome?
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CD4 T Cell
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Do patients with Sjogren Syndrome typically have RF?
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Yes
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In mixed connective tissue disease what ANA level is particularly high?
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Anti-RNP ANA
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People with X-linked Bruton's agammaglobulinemia present with what?
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Recurrent bacterial infections
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What functions are normal in X-linked Bruton's agammaglobulinemia?
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Cell mediated functions
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Sometimes this Ig is the only one deficient in common variable immunodeficiency...
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IgG
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What type of infection is seen in Isolated IgA deficiency?
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Sinopulmonary
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70% of the time hyper-IgM syndrome is inherited how?
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X-linked
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What is a minor characteristic of DiGeorge syndrome?
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Hypocalcemia - hyperactive neurons
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Which chromosome is the deletion on in DeGeorge syndrome?
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22
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What problem does a adenosine deaminase deficiency cause that leads to SCID?
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Accumulation of lymphocyte toxic metabolites
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What is the current therapy for SCID?
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Bone-marrow transplant
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What is the current therapy for WAS?
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Bone-marrow transplant
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What happens with a C1 esterase inhibitor mutation?
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Accumulation of vasoactive kinins
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What type of virus is HIV-1?
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Human type C - non-transforming cytopathic retrovirus
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What does current HIV therapy target?
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Reverse transcriptase and proteases
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Individuals with mutations in _____ are resistant to HIV-1?
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CCR5
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When does HIV transcription/translation take place?
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When the T Cell is activated.
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What is seen with memory T cells and B cells in HIV?
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Selective loss of memory T cells along with activation of polyclonal B cells.
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50% of systemic lymphomas and 100% of CNS lymphomas in AIDS carry what?
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Epstein-Barr Virus genome
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What do you usually not see in mixed connective tissue disease?
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1. Anti-dsDNA antibodies
2. Renal disease |
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What are the three viral enzymes included in HIV-1?
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1. Protease
2. Integrase 3. Reverse transcriptase |
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What is usually associated with the non-branching fibrils of intermediate length in amyloidosis
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The P component
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What type of amyloidosis is associated with chronic inflammation states? What are the plaques made of?
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1. Secondary or Reactive Amyloidosis
2. AA |
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Familial mediterranean fever presents with what type of amyloidosis?
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AA
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Nodular amyloidosis usually involves what type of plaque?
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AL
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Amyloidosis of aging typically presents with what type of plaque?
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Non-mutant transthyretin
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