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219 Cards in this Set

  • Front
  • Back
What type of hypersensitivity casuses anaphylaxis?
Type I
What type of hypersensitivity causes Allergic rhinitis?
Type I
What type of hypersensitivity causes Hemolytic anemia?
Type II
What type of hypersensitivity causes Idiopathic thrombocytopenic purpura?
Type II
What type of hypersensitivity causes Erythroblastosis fetalis?
Type II
What type of hypersensitivity causes Rheumatic fever?
Type II
What type of hypersensitivity causes Goodpasture's syndrome?
Type II
What type of hypersensitivity causes Bullous pemphigoid?
Type II
What type of hypersensitivity causes Graves' Disease?
Type II
What type of hypersensitivity causes Myasthenia gravis?
Type II
What type of hypersensitivity causes SLE?
Type III
What type of hypersensitivity causes Rheumatoid arthritis?
Type III
What type of hypersensitivity causes Polyarteritis nodosum?
Type III
What type of hypersensitivity causes Poststreptococcal glomerulonephritis?
Type III
What type of hypersensitivity causes Serum sickness?
Type III
What type of hypersensitivity causes Arthus reaction?
Type III
What type of hypersensitivity causes Hypersensitvity pneumonitis?
Type III
What type of hypersensitivity causes Type 1 diabetes mellitus?
Type IV
What type of hypersensitivity causes Multiple sclerosis?
Type IV
What type of hypersensitivity causes Guillain-Barre' syndrome?
Type IV
What type of hypersensitivity causes Hashimoto's thyroiditis?
Type IV
What type of hypersensitivity causes Graft-versus-host disease?
Type IV
What type of hypersensitivity causes PPD response?
Type IV
What type of hypersensitivity causes contact dermatitis?
Type IV
What disease is associated with recurrent bacterial infections after 6 mo of age in boys?
Bruton's agammaglobulinemia
What is the genetic defect of Bruton's agammaglobulinemia?
X-linked recessive defect in tyrosine kinase
What cardiovascular presentation would be expected in a pt with DiGeorge syndrome?
Tetany due to hypocalcemia (decreased PTH)
Bruton's agammaglobulinemia is a defect in what cell type?
B Cells
Disease associated with recurrent viral and fungal infections?
DiGeorge syndrome (T cell deficiency)
Associated with 22q11 deletion?
DiGeorge syndrome (T cell deficiency)
In what disease are both B and T cells deficient?
Severe combined immunodeficiency (SCID)
What do pts with SCID present with?
Recurrent viral, bacterial, fungal, and protozoal infections
What causes SCID?
Defect in early stem-cell differentiation
What is the result of an IL-12 receptor deficiency?
Pt presents with dissemenated mycobacterial infections due to decreased Th1 response
What is IgM syndrome?
Defect in CD40 ligand on CD4 T helper cells, inability to class switch. Therefore high IgM, all others low.
What defect causes pt to present with severe pyogenic infections?
IgM syndrome
X-linked defect in ability to mount an IgM response to capsular polysaccharides of bacteria.
Wiskott-Aldrich syndrome
What disease presents with this triad: infections, thrombocytopenic purpura, eczema?
Wiskott-Aldrich syndrome
What immune deficiency presents with increased levels of IgA
Wiskott-Aldrich syndrome
What is Job's syndrome?
Failure of IFN-gamma productions by helper T cells therefore neutrophils fail to respond, presents with coarse facies, cold staph abscesses, retained primary teeth, increased IgE, and dermatologic problems.
What syndrome presents with eczema, increased IgE, and staph abscess?
Job's syndrome
What deficiency presents with recurrent bacterial infections, absent pus formation, and delayed seperation of umbilicus?
Leukocyte adhesion deficiency
Defect in LFA-1 integrin proteins on phagocytes.
Leukocyte adhesion deficiency
What immune cell is deficient in Leukocyte adhesion deficiency?
Phagocyte
What disease presents with recurrent pyogenic infections by staph and strep, partial albinism, and peripheral neuropathy?
Chediak-Higashi disease
What is Chediak-Higashi disease?
Autosomal recessive defect in microtubular function and lysosomal emptying of phagocytic cells.
What immune deficiencies result in decreased production?
Bruton's agammaglobulinemia, DiGeorge syndrome, Severe combined immunodeficiency
What immune deficiencies result in decreased activation?
IL-12 receptor deficiency, IgM syndrome, Wiskott-Aldrich syndrome, Job's syndrome
What immune deficiencies result in phagocytic cell deficiency?
Leukocyte adhesion deficiency, Chediak-Higashi disease, Chronic granulomatous disease
Lack of NADPH oxidase.
Chronic granulomatous disease
What is chronic granulomatous disease?
Defect in phagocytosis of neutrophils due to lack of NADPH oxidase, marked susceptibility to opportunitstic infections.
How do you confirm chronic granulomatous disease?
Nitroblue tetrazolium dye reduction test (doesn't turn blue if they lack NADPH oxidase)
What disease is the nitroblue tetrazolium dye reduction test used to diagnose?
Chronic granulomatous disease
Pt presents with recurrent skin and mucous membrane Candida infections
Either immunocompromised state OR idiopathic chronic mucocutaneous candidiasis
What is chronic mucocutaneous candidiasis?
Idiopathic T cell dysfunction specifically against Candida albicans
What is the most common selective immunoglobulin deficiency?
Selective IgA deficiency, presents with sinus and lung infections, milk allergies, and diarrhea.
Defect in DNA repair enzymes with associated IgA deficiency.
ataxia-telangectasia, presents with cerebellar problems and spider angiomas
Normal numbers of circulating B cells, decreased plasma cells, decreased Ig
Common variable immunodeficiency, defect in B cell maturation
What immune defieincies are idiopathic dysfunctions of B cells?
Selective IgA deficiency, Ataxia-telangectasia, and Common variable immunodeficiency
Antinuclear antibody
SLE
What autoantibodies are associated with SLE?
Antinuclear antibody, Anti-dsDNA, and anti-Smith
Anti-Smith antibody
SLE
Anti-dsDNA antibody
SLE
Antihistone antibody
Drug induced lupus
Anti-IgG antibody
Rheumatoid arthritis
Anticentromere antibody
Scleroderma (CREST)
Anti-scl-70 antibody
Scleroderma (diffuse)
Antimitochondrial antibody
Primary biliary cirrhosis
Antigliadin antibody
Celiac Disease
Anti-basement membrane antibody
Goodpasture's syndrome
Anti-epithelial cell antibody
Pemphigus vulgaris
Antimicrosomal antibody
Hashimoto's thyroiditis
Antithyroglobulin antibody
Hashimoto's thyroiditis
Anti-Jo-1 antibody
Polymyositis, dermatomyositis
Anti-SS-A
Sjogren's syndrome
Anti SS-B
Sjogren's syndrome
What antibody is associated with Drug induced lupus?
Antihistone antibody
What antibody is associated with Rheumatoid arthritis?
Anti-IgG antibody
What antibody is associated with Scleroderma (CREST)?
Anticentromere antibody
What antibody is associated with Scleroderma (diffuse)?
Anti-scl-70 antibody
What antibody is associated with Primary biliary cirrhosis?
Antimitochondrial antibody
What antibody is associated with Celiac Disease?
Antigliadin antibody
What antibody is associated with Goodpasture's syndrome?
Anti-basement membrane antibody
What antibody is associated with Pemphigus vulgaris?
Anti-epithelial cell antibody
What antibody is associated with Hashimoto's thyroiditis?
Antimicrosomal antibody, Antithyroglobulin antibody
What antibody is associated with Polymyositis, dermatomyositis?
Anti-Jo-1 antibody
What antibody is associated with Sjogren's syndrome?
Anti-SS-A, Anti SS-B
Anti-U1 RNP antibody
Mixed connective tissue disease
Anti-smooth muscle antibody
Autoimmune hepatitis
Anti-glutamate decarboxylase
Type I diabetes mellitus
c-ANCA antibody
Wegner's granulomatosis
p-ANCA antibody
Vasculitities other than Wegner's, ex: microscopic polyangitis
What diseases are associated with HLA subtype B27?
PAIR - Psoriasis, Ankylosing spondylitis, Inflammatory bowel disease, Reiter's syndrome
What diseases are associated with HLA subtype B8?
Graves' disease, celiac sprue
What diseases are associated with HLA subtype DR2?
Mutiple sclerosis, hay fever, SLE, Goodpasture's
What diseases are associated with HLA subtype DR3?
Diabetes mellitus type I
What diseases are associated with HLA subtype DR4?
Rheumatoid arthritis, diabetes mellitus type I
What diseases are associated with HLA subtype DR5?
Pernicious anemia due to B12 deficiency, Hashimoto's thyroiditis
What diseases are associated with HLA subtype DR7?
Steroid-responsive nephrotic syndrome
A graft from self
Autograft
What is an allograft?
Graft from nonidentical individual of same species
Graft from identical twin or clone?
Syngeneic graft
What is a xenograft?
Graft from different species
What is the MOA of cyclosporine?
Binds to cycophilins, the complex inhibits calcineurin, stopping IL-2 production (end result blocks clonal expansion of T cells)
What is the clinical use of cyclosporine?
suppress organ rejection, some autoimmune disorders
What is an alternative to cyclosprine that is less toxic and more potent?
Tacrolimus
What drug binds FK-binding protein and inhibits IL-2 production?
Tacrolimus
Antimetabolite precursor of 6-mercaptopurine, toxic to proliferating lymphocytes
Azathioprine (cytotoxic agent)
What are the toxic effects of Azathioprine?
Bone marrow suppression, can be increased if taken with allopurinol
Monoclonal antibody that binds to CD3 on T cells used for immunosuppression after kidney transplatation.
Muronomonab-CD3
What is the MOA of Sirolimus?
Binds mTOR(mamallian target of rapamycin), inhibits T cell proliferation in response to IL-2.
What are the toxic effects of Sirolimus?
Hyperlipidemia, thrombocytopenia, and leukopenia
What immunosuppressant inhibits de novo guanine synthesis?
Mycophenolate mofetil
What is the MOA for Daclizumab?
Monoclonal antibody with high affinity for the IL-2 receptor on activated T cells.
What are the toxic effects of Cyclosporine?
Predisposes pts to viral infections and lymphoma, nephrotoxic.
What are the toxic effects of Tacrolimus?
Nephrotoxicity, peripheral neuropathy, hypertension, pleural effusion, and hyperglycemia
What is Aldesleukin (IL-2) used for clinically?
Renal cell carcinoma, metastatic melanoma
What recombinant cytokine might you give a pt for anemia, especially associated with renal failure?
Erythropoietin (epoetin)
What is Filgrastim?
Granulocyte colonys-stimulating factor, used for recovery of bone marrow
What is Sargramostim?
Granulocyte macrophage colony stimulating factor, used for recovery of bone marrow
What recombinant cytokines might you give a pt for recovery of bone marrow?
Filgrastim, Sargamostim (Granulocyte colony stimulating factors)
What is the clinical use of alpha-interferon?
Hepatitis B and C, Kaposi's sarcoma, leukemias, malignant melanoma
What recombinant cytokine is used clinically for Kaposi's sarcoma?
alpha-interferon
What recombinant cytokine is used clinically for Hepatitis B and C?
alpha-interferon
What is beta-interferon used for clinically?
Multiple sclerosis
What recombinant cytokine is used clinically for Multiple sclerosis?
beta-interferon
What is the clinical use of gamma interferon?
Chronic granulomatous disease
What recombinant cytokine is used clinically for Chronic granulomatous disease?
gamma-interferon
What is the clinical use of Oprelvekin (IL-12)?
Thrombocytopenia
What is the clinical use of Thrombopoietin?
Thrombocytopenia
What recombinant cytokine is used clinically for Thrombocytopenia?
Oprelvekin (IL-11), Thrombopoietin
What recombinant cytokine is used clinically for malignant melanoma?
alpha-interferon
What recombinant cytokine is used clinically for leukemias?
alpha-interferon
What part of antibody recognizes antigens
Variable part of L and H chains
What types of antibody fix complements
The Fc regions of IgM and IgG
What portions of the antibody does heavy chain contribute to
Both Fab and Fc regions
What are the characteristics of the Fc region
Constant, Carboxy terminal, Complement binding (IgG + IgM), Carbohydrate side chains
What are some benefits of the antibody
(1) Promote opsonization (2) Prevent bacterial adherence to cells (3)Activate complement and enhances opsonization and lysis
What factors are responsible for antibody diversity
(1) Random recombination of VJ (light-chain) or VDJ (heavy-chain) regions (2) Random recombination of heavy chains and light chains (3) Somatic hypermutation (4) Addition of nucleotides to DNA during recombination by terminal deoxynucleotidyl transferase
What types of antibodies to mature B lymphocytes express on their surface
IgM and IgD. These may differentiate by isotype switching (mediated by cytokines and CD 40 ligands) into plasma cells that secrete IgA, IgE, or IgG
What are the characteristics of IgG
(1) The main antibody in secondary response (2) Most abundant (3) Fixes complement (4) Opsonizes bacteria (5) Neutralizes bacteria toxins and viruses (6) Crosses the placenta
What are the characteristics of IgA
(1) Prevents the attachment of bacteria and viruses to mucus membrane (2) Does not fix complement (3) Is a monomer of a dimer (4) Found in secretions (5) Picks up secretory component from epithelial cells before secretion
What are the characteristics of IgM
(1) Produced in the primary response to an antigen (2) Fixes complement (3) Does not cross the placenta (4) Is the antigen receptor on the surface of B Cells (5) Is a monomer on B Cell or Pentamer
What are the characteristics of IgD
(1) Found on the surface of many B Cells and in serum (2) Its function is unclear
What are the characteristics of IgE
(1) Mediates immediate (Type 1) hypersensitivity by inducing the release of mediators from mast cells and basophils when exposed to allergen (2) Mediates immunity to warms by activating eosinophils (3) Has the lowest concentration in serum
What is an allotype (Polymorphism)
An Ig epitope that differs among members of the same species. Can be on a light chain or heavy chain. (ALLotypes represent different ALLeles
What is an Isotype
An Ig epitope common to a single class of Ig. There are 5 classes of Igs (IgA, IgD, IgE, IgG, IgM) and the difference is determined by the heavy chain. Isotype = iso (same); common to same class
What is an idiotype
An Ig epitope determined by antigen binding site. It is specific to a given antigen. (Idiotype = idio (unique); hypervariable region is unique)
What are the characteristics of IL-1
(1) Secreted by macrophages (2)Cause acute inflammation (3) Induces cytokine production to recruit leukocytes (4) Activates endothelium to express adhesion molecules (5) Is an endogenous pyrogen
What are the characteristics of IL-2
(1) Secreted by Th Cells (2) Stimulates grow of helper and cytotoxic T Cells. (IL-2: Stimulates T Cells)
What are the characteristics of IL-3
(1) Secreted by activated T Cells (2) Supports the growth and differentiation of bone marrow stem cells (3) Has a function similar to GM-CSF. (IL-3: Stimulates bone marrow growth)
What are the characteristics of IL-4
(1) Secreted by Th2 Cells (2) Promotes growth of B Cells (3) Promotes class switching to IgE and IgG. (IL-4: Stimulates IgE production)
What are the characteristics of IL-5
(1) Secreted by Th2 Cells (2) Promotes differentiation of B Cells (3) Promotes class switching to IgA (4) Stimulates production and activation of eosinophils. (IL-4: Stimulates IgA production)
What are the characteristics of IL-6
(1) Secreted by Th cells and macrophages (2) Stimulates production of acute phase reactants and immunoglobulins
What are the characteristics of IL-8
(1) Secreted by macrophages (2) Major chemotactic factor for neutrophils. ('Clean up on aisle 8' - Neutrophils are recruited by IL-8 to clear infections)
What are the characteristics of IL-10
(1) Secreted by regulatory T Cells (2) Inhibits action of activated T Cells
What are the characteristics of IL-12
(1) Secreted by macrophages and B Cells (2) Activates NK and Th1 Cells
What are the characteristics of gamma interferon
(1) Secreted by Th1 cells (2) Stimulates macrophages
What are the characteristics of TNF
(1) Secreted by macrophages (2) Mediated septic shock (3) Causes leukocyte recruitment and vascular leak
What cell surface proteins are on Helper T Cells
CD4, TCR, CD3, CD28, CD40L
What Cell surface proteins are on Cytotoxic T Cells
CD8, TCR, CD3
What Cell Surface proteins are on B Cells
IgM, B7, CD19, CD20, CD27, CD40, MCH II
What Cell surface proteins are on macrophages
MCH II, B7, CD40, CD14. Receptors for Fc and C3b
What Cell surface proteins are on NK Cells
Receptors for MHC I, CD 16, CD56
What Cell surface receptor is on all cells except mature red cells
MHC I
What is the definition of Complement
Complement is a system of proteins that interact to play a role in humoral immunity and inflammation
What is the function of the Membrane attack complex of complement
To defend against gram negative bacteria
How is complement activated
(1) Complement is activated in the Classic pathway by IgG and IgM. (GM makes classic cars) (2) Complement is activated by molecules on the surface of microbes (especially endotoxins) in the alternative pathway
What are the primary opsonins in bacterial defense
C3b and IgG (C3b = opsonization - Bind Bacteria)
How is complement activation in self-cells prevented
By Decay Accelerating Factor (DAF) and C1 esterase inhibitor
What is C3a and C5a known for
Anaphylaxis
What is a function of C5a
Neutrophil chemotaxis
What is the function of the C5b - 9 complement
Cytolysis by membrane attack complex (MAC)
What causes hereditary angioedema
Deficiency of C1 esterase inhibitor
What is the problem associated with C3 complement deficiency
Severe, recurrent pyogenic sinus and respiratory tract infections
What is the problem associated with C6 - C8 complement deficiency
Neisseria bacteremia
What is the problem associated with deficiency of Decay accelerating Factor (DAF)
Complement-mediated lysis of RBC and paroxysmal nocturnal hemoglobinemia (PNH)
What interferon place an uninfected cell in an antiviral state
Interferons alpha, beta, and gamma
What is the function of interferons
(1) Interferons induce the production of a ribonuclease that inhibits viral protein synthesis by degrading viral mRNA (but not host mRNA) (2) They activate NK Cells to kill virus-infected cells - Interferons INTERFERe with viruses
What is the function of interferons alpha and beta
They inhibit viral protein synthesis
What is the function of interferon gamma
They increase MHC I and II expression and antigen presentation in all cells
What are the characteristics of ACTIVE immunity
(1) Induced after exposure to foreign antigens (2) Slow onset (3) Long-lasting protection (memory)
What are the characteristics of PASSIVE immunity
(1) Based on receiving pre-formed antibodies from another host (2) Rapid onset (3) Short lifespan of antibodies (half-life = 3 weeks) - After exposure of Tetanus Toxin, Botulinum Toxin, HBV, or Rabies, patients are given preformed antibodies (passive) - To Be Healed Rapidly
What are some examples of antigen variations in bacteria
(1) Salmonella has two flagella variants (2) Borrelia has relapsing fever (3) Neisseria gonorrhoeae has pilus protein
What are some examples of antigen variations in virus
(1) Influenza (major variation = shift, minor variation = drift)
What are some examples of antigen variations in parasites
Programmed rearrangement in Trypanosomes
What are some examples of mechanisms of antigen variation
DNA Rearrangement and RNA segment reassortment (e.g. influenza major shift)
What is anergy
Anergy is a condition where self-reactive T Cells become non-reactive without co-stimulatory molecule. B Cells also become anergic, but tolerance is less complete than in T Cells
Anaphylactic and Atopic hypersensitivity
Hypersensitivity Type I - Free antigen cross-links IgE on presensitized mast cell and basophils, triggering release of vasoactive amines (i.e. histamine). Reaction develops rapidly after antigen exposure due to preformed antibody. (First and Free (anaphylaxis))
Antibody-mediated hypersensitivity
Hypersensitivity Type II - IgM or IgG bind to fixed antigen on enemy cell, leading to lysis (by complement) or by phagocytosis - Cyt-2-toxic. Antibody and Complement lead to membrane attack complex (MAC)
Delayed (T-Cell mediated) type hypersensitivity
Hypersensitivity Type IV - sensitized T lymphocytes encounter antigen and then release lymphokines (leads to macrophage activation). 4th and last = delayed. Cell-mediated, so not transferable by serum. 4T's = T lymphocytes, Transplant rejections, TB skin tests, Touching (contact dermatitis)
Mnemonic for Hypersensitivity Reactions
ACID = Anaphylactic and Atopic (Type I), Cytotoxic - antibody-mediated (Type II), Immune complex (Type III), and Delayed - cell mediated (Type IV)
An immune complex disease in which antibodies to the foreign protein are produced
Serum Sickness - Type III hypersensitivity. Takes 5 days. Immune complexes form and are deposited in membranes, where they fix complement (leads to tissue damage). More common than Arthus reaction
Antigen-antibody complexes activate complement, which attracts neutrophils; neutrophils release lysosomal enzymes
Immune complex - Type III hypersensitivity
A local subacute antibody-mediated hypersensitivity reaction. Characterized by edema, necrosis, and activation of complement
Arthus reaction = Type III hypersensitivity. Intradermal injection of antigen induces antibodies, which form antigen-antibody complexes in the skin.
Imagine 3 things stuck together: Antigen-Antibody-Complement. Most serum sickness is now caused by drugs, not serum. Characterized by fever, urticaria, arthralgia, lymphadenopathy, proteinuria 5 - 10 days after exposure to antigen. Antigen-Antibody complexes cause the Arthus reaction
Type III hypersensitivity
What occurs in a lymph node follicle?
B-cell localization and proliferation
What occurs in a lymph node Medulla?
Communicate with lymphatics, contains reticular cells and macrophages
What occurs in a lymph node Paracortex?
Houses T cells; enlarges in an immune response; not well developed in Di George
Spleen-Periarterial lymphatic sheath (red pulp)
T cell location in spleen
Spleen-Follicles (white pulp)
B-cell localization
Site of T-cell maturation?
Thymus
Site of B-cell maturation?
Bone Marrow
What is innate immunity?
Receptors that recognize pathogens encoded in the germline. Response is fast and nonspecific with no memory. (PMN, Macrosphages, NK cells, complement)
What is adaptive immunity?
Receptors that undergo recombination during lymphocyte development; has memory (T cells and B cells, Antibody)
MHC
Major histocompatibilty complex encoded by Human Leukocyte Antigen genes (HLA)
MHC I-Location
All nucleated cells, loaded in RER, mediates viral immunity
MHC II- Location
Antigen presenting cells, loaded in acidified endosome
MHC II- Types
HLA-DR, HLA-DP, HLA-DQ
B-cell function
Make antibody
T-cell function-CD4+
CD4+ T cells help B cells make antibody, and produce gamma-interferon
T-cell function-CD8+
Kill virus-infected cell
Natural killer cells-Killing mechanisms
Uses perforin and granzymes to induce apoptosis
How to activate a NK cell?
IL-12, INF-beta, INF-alpha
What is a superantigen?
Cross-links Beta-region of TCR to the MHC class II on APC
How many signals are required for T and B cell activation?
Two
T cell activation-Helper
1)Antigen on MHC II and Costimulatory (B7-CD28)
B cell activation
1) IL-4, IL-5, IL-6 2) CD40 and CD40 ligand on Th Cell
T-cell activation- Cytotoxic
1)TRC-MHC I (with antigen) 2)IL-2 from Th cell