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151 Cards in this Set
- Front
- Back
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Lymph Node Functions
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Storage of B and T cells
Activation of B and T Cells Antibody Production Nonspecific Mac Filtration |
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Follicle
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Site of B cell proliferation
This is in the cortex Primary Follicles are dense and dormant Secondary Follicles have a pale central germinal centers and are active |
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Medulla
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consists of cords and sinuses
the sinuses communicate with the efferent lymphatics and contain reticular cells and macrophages Medullary cords are closely packed lymphocytes and plasma cells |
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Where are the macrophages in a lymph node?
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The medullary sinus
They also have reticular cells |
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Where are the B and T cells in a lymph node?
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B cells are in the follicle
T cells are in the paracortex |
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Where are plasma cells in a lymph node?
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Medullary cords
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What is the paracortex?
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Houses the T cells
This is between the follicle (holding B cells) and the medulla |
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Where are the B cells located in the spleen?
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White Pulp, in the follicles
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Where are the T cells located in the spleen?
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In the Red Pullp, and Periarteriolar Lymphatic Sheath
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What is the asplenic person susceptible to?
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Encapsulated bateria
ie. Strep Pneumo, H. Influenza, Salmonella |
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Where are plasma cells in a lymph node?
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Medullary cords
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What is the paracortex?
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Houses the T cells
This is between the follicle (holding B cells) and the medulla |
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Where are the B cells located in the spleen?
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White Pulp, in the follicles
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Where are the T cells located in the spleen?
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In the Red Pullp, and Periarteriolar Lymphatic Sheath
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What is the asplenic person susceptible to?
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Encapsulated bateria
(tuberculosis) |
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Why are you susceptible to encapsulated organisms in splenic dysfunction?
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you have decreased IgM, which means you have less complement activation, less C3b opsonization
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What type of cells would you expect to see post-splenectomy?
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Howell Jowell Bodies
Target Cells Thrombocytosis |
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What is the Thymus?
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Site for T cell differententiation and maturation
It is encapsulated, and arises from the 3rd branchial pouch lymphocyes are mesechymal origin |
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What cytokine will induce a T helper cell into a Th1
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IL-12
That Th1 cell will then produce IL-2, and IFN gamma |
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What cytokine will induce a T helper cell into a Th2
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IL-4
That Th2 cell will then produce IL-4, IL-5, and IL-10 and the Th2 cell will help the B cells make antibody, more IgE than IgG IFN gamma (produced by Th1) will inhibit Th2 mediated processes |
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What are some functions of IFN gamma
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recruits macrophages, inhibits Th2 mediates processes
released by Th1 cells, thus the key mediator in delayed type hypersensitivty activated macrophages express more MHC II molecules to facilitate further APC, they secrete PDGF to stimulate fibroblast proliferation and collagen sysnthesis, they secrete TNF, IL-1 and chemokines to promote inflammation, AND they produce MORE IL-12 to promote more Th1 and thus IFN gamma production that were induced by what? (IL-12) |
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Where will you find MHC I?
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on all nucleated cells (these are the HLA-I-A, B, C)
the antigen is located in the RER and this mediates viral immunity it pairs with B2 microglobulin to aid in transport to the cell surface |
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Where will you find MHC II?
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Only on antigen presenting cells
and the antigen is loaded following the release of the invariant chain in an acidified endosome |
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What are the major functions of B cells?
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To make antibody
IgG: opsonize bateria and neutralize viruses |
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What are the major functions of T cells?
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CD4+ T cells help B cells to make antibody and produce gamma interferon, which activates macrophages
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Hypersensitivity reactions
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B cells mediate I, II, and III
T cells mediate IV |
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Organ rejection
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Hyperacute are done by antibodies (B cell mediated)
Chronic and Acute (T cell) |
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Type I Hypersensitivity
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B cell
immune response releases vasoactive and spasmogenic substances that act on blod vessles and smooth muscle proinflammatory cytokines recruit inflammatory cells Examples: Anaphylaxis, Allergies, Bronchial Asthma, Atopic Dermatitis produce IgE antibodies, mast cell degranulation, and recruitment of inflammatory cells This causes vasodilator, smooth muscle constriction, edema, mucous production and inflammation First phase: within 5- 30 minutes (edema, vasodilation, smooth muscle spasm, and contraction Second phase: sets in 2-24 hours later, infiltration with eosinophils, neutrophils, basophils, monocytes etc |
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Type II Hypersensitivity
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Antibody mediated (IgG and IgM)
Exmaples: Autoimmune hemolytic anemia, goodpasture syndrome, myasthenia gravis, graves disease, pemphigus vulgaris, drug reactions in which antibodies are produced that react with the drug that may be attached to the surface of the erythrocyte or other cells mediated by antibodies directed toward antigens present on cell surfaces or extracellular matrix The effector mechanism, mainly complement and macrophages are used after the antibody binds to the cell surface receptor. |
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Type III Hypersensitivity
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Immune Complex, where antibody/antigen complexes elicit damage and inflammation by settling in tissues
SLE, Reactive Arthritis, Rheumatoid A, post-Strep Glomerulosclerosis, Polyarteritis nodosa, Serum Sickness, Arthus reaction, Acute Glomerulonephritis |
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Type IV hypersensitivity
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both delayed type (CD4 and Th1 dependent) and cytotoxic (CD 8)
Contact dermatitis, rheumatoid arthritis, multiple sclerosis, type I diabetes, tuburculin, guillane barre |
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What happens when an individual is first exposed to tuburculin protein antigens?
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antigen presenting cells will show the antigen to CD4 T cells, this drives the CD4 t cell to differentiate into Th1 cell, The Th1 cells enter circulation and remain there for years, on administration of tuberculin, the Th1 cells recognize and are activated, secreting IL-2 and IFN gamma which is the big macrophage activator...
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What is an ACTIVATED macrophage?
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has more MHC II cells expressed to faciliate APC, secretes PDGF to stimualte fibroblast proliferation and collagen synthesis, secretes IL-12 which will drive a Th1 prduction to make even more IFN gamma to activate macrophages, big cycle
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What does TNF do?
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along with lymphotexin, they Exert effects on the endothelial cells
increase prostacyclin synthesis to favor vasodilation increased P-E selectin expression to faciliate extravasation Induction and secretion of chemokines like IL8 |
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hyperacute rejection
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antibody mediated, occurs within minutes to hours
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What is acute rejection?
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occurs within days of transplantation, T cell mediated
acute cellular rejection: within months, see elevated serum creatinine levels (signs of renal failure) acute humoral rejection: rejction vasculitis |
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Chronic Rejection
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progressive rise in serum creatinine, over 4 to 6 months
interstitial fibrosis, vascular changes, atrophy etc |
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Cyclosporine
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mainstay of immunosuppression in transplants
blocks transcription factor (TF of activated T cells) to prevent transcription of cytokines, particulary IL-2 |
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What CD marker are on macrophages?
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CD 14
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Whatis required for Th cell activation?
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MHC II has to present the antigen to a Th cell, along with with B7 costimulus.
The Th cell's TCR binds to the antigen/MHCII complex, and the CD 28 reacts with the APC's B7 stimulus. This activates the Th cell to produce cytokines |
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What is required for B cell class switching?
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The Th2 cell must secrete IL4, 5 or 6
Along with the CD40 receptor activation when it bings the CD40Ligand on the Th cell So the Th2 cell must have the CD40ligand out, along with secreting IL4,5, or 6 to activate the B cell to make a switch in class |
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How do you get the Tc to activate?
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MHC I presents endogenously synthesized proteins (either viral or self) and this is recognized by the TCR receptor on the Tc cell.
Th1 secretes IL2 which will activate the Tc cell to kill the virus infected cell |
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What fixes complement?
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The Fc portion of IgG and IgM
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How do you generate antibody diversity?
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1. random recombination of VJ (light chain) or V(D)J (heavy chain) genes
2. Random combination of heavy with light chains 3. Somatic hypermutation (following antigenic stimulation) 4. Addition of nucleotides to DNA during recombination by terminal deoxynucleatide transferase |
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What is opsonization?
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Antibody binds to the antigen, and promotes phagocytosis
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What is neutralization?
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The antibody binds to the bacteria, preventing bacteria from adhering to the cell membrane
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What is complement activation?
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The antibody (Fc portions of IgG and IgM) activates complement, enhancing opsonization and lysis
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What do mature B lymphocytes express on their cell surface?
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IgM and IgG---> they may differentiate by isotype switching (alternative splicing of mRNA; mediated by cytokines and CD40Ligand) and differentiate into plasma cells that can secrete IgA, IgE and IgG
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What is an Ig epitope?
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allotype: different allelles, vary between individuals of the same species
isotype: IgG or IgA or IgM (5 classes, determined by their heavy chain) idiotype: specific for a particular antigen (hypervariable region) |
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What makes up the innate immunity?
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epithelial barriers, phagocytes, NK cells, and complement
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What makes up the adaptive immunity?
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lymphocytes and their products, antibodies
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How does the innate immune system recognize pathogens?
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mannose residues and N-formylmethionine
toll like receptors are on the lymphocyte and one activated they stimulate nfkappaB to produce cytokines to promote the microbial properties of of phagocytes |
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How do phagocytes kill microbes?
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envelope them into the hydrolytic vesicles where the microbes are destroyed by reactive oxygen and nitrogen intermediates, and hydrolytic enzymes
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How is complement activated though the innate system? adaptive?
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Innate: alternative or lectin pathways
Adaptive: classical |
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What are the types of adaptive immunity?
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cell mediated and humoral mediated
cell mediated: defense against intracellular antigens (viral)--> T cell mediated Humoral mediated (defense against extracellular microbes) (bacteria)--> B cell mediated |
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Where are mature, naive T cells?
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In the blood, they constitute 60-70% of lymphocytes and in the T-cell zones of peripheral lymphoid organs
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What is required for induction of cell-mediated immunity?
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presentation of a processed, membrane bound antigen by an antigen presenting cell to a T cell
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Tell me about TCRs
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rearrangement of the TCR genes occurs in the thymus, so each T cell has a different TCR receptor
each TCR is linked to the CD3 receptor on the surface of T cells |
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Where is B7?
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On the antigen presenting cell; it binds the CD28 marker on T cells, needed to activate the T cell on presentation
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What is the function of the CD4 cell?
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the master regulator; HIV destroys this cell
th1: produces IL-2 and IFNgamma th2: IL4, 5, and 13 |
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What is the function of the B cell?
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they constitute about 10 to 20% of the circulating peripheral lymphocytes (lymph nodes in the superficial cortex, in the spleen, in the white pulp)
They recognize IgM and IgD are present on the surface of B cells, and the B cell recognizes antigen through their IgM and IgD. After antigenic stimulation, the B cells form plasma cells that secrete immunoglobulins which mediate HUMORAL immunity |
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What does EBV infect?
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B cells, because the CD21 receptor
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What do secretory antibodies do?
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enter mucosal secretions and blood and they are able to find, neutralize and eliminate antigens
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what are the opsonizers?
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C3b and IgG
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leukocyte adhesion deficiency syndrome
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defect in LFA-1 (CD-18) on phagocytes
presents early with bacterial infections, ABSENT pus formation, neutrophilia and delayed separation of the umbilicus! the little lad couldn't get rid of his umbilical cord! |
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chediak-higashi syndrome
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autosomal recessive
defect in microtubular function and lysosomal empyting of phagocytic cells. Recurrent pyogenic infections by staph or strep, partial albinism, neuropathies, (nystagmus) |
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Chronic Granulomatous Disease
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Lack of NADPH oxidase, making for defective neutrophil microbicidal activity.
marked susceptibility to opportunistic infections with bacteria (particularly S. Aureus, e. coli, and asperigillus) Diagnosis confirmed with a negative nitoroblue tetrazolium dye test-->can't form H202 |
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Chronic Mucocutaneous Candidiasis
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T cell dysfunction, especially against candida albicans
presents with skin and mucous membrane infections |
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Selective Immunoglobin Deficiency
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deficiency in a specific type of immunoglobin, possibly due to a defect in switching.
Generally IgA deficiency is the most common-- presents with sinus and lung infection. Milk allergies and diarrhea are common. Anaphylaxis upon exposure to IgA blood products. |
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What IL stimulates IgE?
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IL-4
HOT TBone stEAk |
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What IL stimulates bone marrow?
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IL-3
secreted by activated T cells |
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What IL is important for IgA class switches?
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IL-5
promotes the differentiation of B cells, and stimulates the production and activation of eosinophils as well |
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What is a superantigen?
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S. Aureus and S. Pyogenes
Cross link the beta region of the T cell receptor to the MHC II class on the APC--> resulting in an uncoordinated release of IFN gamma from Th1 cells, and subsequent release of IL-1, IL-6 and TNF alpha from macrophages cross link Beta region of TCR to the MHC II unregulated release of INF gamma (th1), IL-1, IL-6 and TNF alpha from macs |
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How do endotoxins/LPS work?
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seen on all gram negative bacteria--> this can DIRECTLY stimulate the CD14 receptor on macrophages-- no Th cells involved
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Where is B7 located?
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on the APC
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where is CD28?
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On T cells
binds with the B7 on APCs and is needed for T cell activation |
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What is required for Tcytotoxic activation?
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Need IL-2 from the Th1, to activate the cytotoxic cell to kill a virus infected cell
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What parts of complement are needed for viral neutralization?
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C1-4
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What activates the classical complement pathway?
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IgM and IgG complexes with an antigen--> the Fc portions are complement fixers
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What parts of complement are responsible for anaphylaxis?
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C3a and C5a
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What is hereditary angioedema?
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A deficiency of C1 esterase
(don't give an ACE inhibitor!) |
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What would a deficiency of C3 look like?
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Recurrent pyogenic sinus and respiratory tract infections
with increased susceptibility to type III hypersensitivity reactions |
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When does IgM synthesis begin? IgG?
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At Birth-- presence of IgM at birth may indicate an infection (CMV)
IgG synthesis begins 2 months after birth (presence of IgG at birth is maternally derived) |
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What codes for MHC-II proteins?
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HLA-DP, DR, DQ
recognized by CD4 cells |
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What HLA is associated with MS? Anylosing Spondylitis? Type I diabetes?
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MS: HLA-DR2
AS: HLA-B27 DM1: HLA-DR3 and DR4 |
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What do NK cells look like?
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Large granular lymphocytes in the peripheral blood
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Type I hypersensitivity reaction?
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On first exposure, APC process the antigen, and present it to Th2 helper cells, which produce IL-4 causes the class switch from IgM to IgE
IL-5 causes the activation of eosinphils On second exposure, senstized IgE are bound to mast cells, once they bind and cross link, we see degranulation of mast cells Mast cells are active early and late in the response Early: release histamine, chemotactic factors for eosinophils and proteases--> causes tissue swelling and bronchoconstriction Late: the mast cells synthesize and release prostaglandins and leukotrienes to ENHANCE and PROLONG the acute inflammatory response |
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How would you evaluate a kid with eczema?
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type I hypersensitivity
scratch test: best SENStivitiy positive test: wheal and flare reaction after introducing the antigen Radioimmunosorbent test: detects specific IgE antibodies in the serum that are antigen specific allergens |
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type II hypersensitivity?
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antibody dependent
complement dependent: lysis: by the MAC complex (need IgG or IgM to fix complement) phagocytosis:fixed macrophages (as in the spleen) phagocytose hematopoetic stem cells (RBCs) coated by IgG antibodies and or complement complement independent: |
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How would you evaluate type II hypersensitivity?
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Direct or Indirect Coombs test
Direct: detects IgG and or C3b attached to RBCs Indirect: detects the antibodies in the serum (anti-IgD) |
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How does type III hypersenstivity start?
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fisrt exposure: synthesis of antibodies (IgG)
Second exposure: IgG, Antibody-Complement deposition complement activation, C5a which attracts neutrophils and damages tissues TEST: immunofluresce the tissue to stain for the complexes (glomerulonephritis) |
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Farmer's Lung
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type III hypersensitivity, Arthus reaction
localized immunocomplex reaction to thermophilic actinomyces breathed into the lungs |
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What are you trying to confirm by using a patch test?
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Type IV hypersensitivity reaction
CONTACT DERMATITIS |
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Hyperacute Rejection
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Type II hypersensitivity
irreversible reaction within minutes ABO incompatibility with blood types. |
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SCID
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multiple causes: ADA deficiency, failure to synthesize MHC II antigens, defective IL-2 receptors (most common form, X-linked)
No T or B cells |
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Bruton's Agammaglobunemia
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X- linked recessive, defect in BTK (tyrosine kinase gene)
low levels of all immunoglobulins, low B cells, recurrent BACTERIAL infections |
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Job's Syndrome
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Failure of Neutrophils
Failure of IFN gamma production by helper T cells, therefore the neutrophils can't respond (no chemotactic stimuli) Present with COARSE FACIES, Staphlococcal Abscesses, retained Primary Teeth, Increased IgE, and dermatological problems (FATED) |
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CVID
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normal numbers of circulating B cells, decreased plasma cells, defect in B cell maturation, decreased Ig, increased risk of autoimmune and lymphoma
what is the difference between bruton's and CVID? CVID there are normal NUMBERS of B cells, Bruton's not. |
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Anticentromere Antibody
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CREST, Scleroderma
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Anti-Scl-70
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Scleroderma (DIFFUSE)
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Antigliadin and antiendomysial antibodies
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Seen in Celiac Disease
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Anti-basement membrane
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Seen in Goodpasture's Syndrome
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What autoantibody would you see with Hashimoto's Thyroditis?
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Anti-microsomal and anti-thyroglobulin
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Anti-Jo-1
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Polymyositis and Dermatomyositis
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Anti-U1 RNP
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Mixed Connective Tissue Disorder
Ribonucleoprotein |
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Anti-Glutamate Decarboxylase
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seen in type I diabetes mellitus
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Anti-smooth muscle
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Autoimmune hepatitis
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c-ANCA
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Wegener's Granulomatosis
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p-ANCA
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other vasculitides
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What HLA subtype is associated with Rheumatoid Arthritis? Pernicious Anemia? Grave's Disease?
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RA: DR-4 (also for DM1)
Pernicious Anemia (Vb12 deficiency) see DR5 |
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What is an allograft?
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from nonidentical individual of the same species
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Cyclosporine
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complex blocks the differentiation and activation of T cells by inhibiting calciuneurin, thus preventing the production of IL-2 and it's receptor
This is nephrotoxic: but preventable with mannitol diureisis |
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Tacrolimus (FK506)
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Similar to cyclosporine, binds to the FK binding protein, inhibiting the secretion of IL-2 and other cytokines
it is a potent immunosuppresant with significant toxicity nephrotoxic, peripheral neuropathy, HTN, pleural effusion and hyperglycemia |
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Azathioprine
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antimetabolite precursor to 6 mercaptopurine that interferes with the metabolism and synthesis of nucleic acids--> toxic to proliferating lymphocytes
this is used for kidney transplants and autoimmune diseases (glomerulonephritis and hemolytic anemia) causes bone marrow suppression, xanthine oxidase metabolizes the active metabolite of 6mercaptopurine, thus allopurinol increases the toxic effects of azathioprine |
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Muronomab--CD3 (OKT3)
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This is a monoclonal antibody that binds to the CD3 receptor, the episilon chain, on the surface of T cells, This blocks cellular interaction with CD3--
toxicity causes cytokine release syndrome and hypersensitivity reactions |
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Sirolimus (rapamycin)
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binds to mTOR, inhibits T cell proliferation in repsonse to IL-2
clinically used alongside cyclosporine and corticosteroids after a kidney transplant Toxicity: hyperlipidemia, thrombocytopenia and leukopenia |
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Mycophenolate Mofetili
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Inhibits de novo guanine synthesis and blocks lymphocyte production
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Daclizumab
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Monoclonal antibody with high affinity to the IL-2 receptor on activated T cells
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Aldesleukin
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Renal Cell Carcinoma, Metastatic Melanoma
This is a recombinant IL-2 |
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Filgrastim
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recombinant G-CSF
used to recover bone marrow |
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Sargramostim
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recombinant Granulocyte Macrophage Colony Stimulating Factor-- used for the recovery of bone marrow
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alpha interferon recombinant
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used for Hep B and C, Kaposi's Sarcoma, Malignant Melanoma, and leukemias
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Beta Interferon Recombinant
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used for MS
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Gamma Interferon
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Used for Chronic Granulomatous Disease
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What recombinant cytokines would you give to someone with thrombocytopenia?
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Oprelvekin (IL-11) and
Thrombopoetin |
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What is the function of alpha and beta inferon?
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They inhibit VIRAL protein synthesis
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What is the function of gamma interferon?
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increase the expression of MHC I and II on all cells to faciliate antigen presentation
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You have a negative NTB test, what do you do?
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Diagnose Chronic Granulomatous Disease
your patient likely has recurrent staphlococcal infections, alongside opportunistic infections like asperigillus and E. Coli. Tx with gamma interferon (this will enhance antigen presentation by all cells) |
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For what pathogens would you give pre-formed antibodies to induce passive immunity?
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To Be Healed Rapidly
Tetanus Toxoid Botulium toxin HBV Rabies |
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What is DAF?
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Decay Accelerated Factor (alongside C1 esterase) it prevents complement activation on self-cells
DAF deficiency (which is a GPI-anchored enzyme) leads to complement mediated lysis of RBCs and paroxysmal nocturnal hemoglobinuria |
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DAF deficiency
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PNH and hemolytic anemia
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What is anergy?
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Self reactive T cells become nonreactive without the costimulatory molecule.
B cells also become anergic, but tolerance is less complete than with T cells |
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Acute Rejection
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The most common type of rejection
occurs within days to weeks Type IV and II hypersensitivity see extensive interesitial round cell lymphocytic infiltrates in the graft, edema and endothelial cell injury |
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What increased risk do you endure taking immunosuppressant therapy (like cyclosporine, OKT3 or corticosteroids)
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increased risk of SCC of the skin (most commonly)
malignant melanoma squamous cervival cell cancer |
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Of the types of rejection, which are reversible?
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Acute
Hyperacute and Chronic are irreversible |
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What do you see with Graft versus host disease?
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jaundice (bile duct necrosis), diarrhea (GI mucosal ulcerations), dermatitis
the donor T cells recognize the host to be foreign, activates host |
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Kidney transplants
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better survival from a living donor than from a cadaver
note: in a bone marrow transplant, the host assumes the donors blood type; danger of GVHD and CMV infection |
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SLE
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Hematologic Findings:
autoimmune hemolytic anemia, thrombocytopenia and leukopenia Lymphatics: splenomegaly and generalized painful lymphadenopathy Musculoskeletal: small joint inflammation with absence of joint deformity Skin: liquefactive degeneration of immune complex deposition along the BM, malar rash Renal: Diffuse proliferative glomerulonephritis ( most common glomerulonephritis) Cardiac:Fibrinous Pericarditis with effusion (most common SLE cardiac finding) may also see Libman Sacks endocarditis (sterile vegetations on the mitral valve) |
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Libman Sacks Endocarditis
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sterile vegetations on the mitral valve, seen in SLE
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What is the most common drug implicated in drug-induced SLE.
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Procainamide-- and you'll see anti-histone antibodies
Hydralazine is also implicated With drug induced lupus, there is low incidence of renal or CNS involvement, symptoms disappear with discontinuance of the drug, and you'll see ANTI-HISTONE antibodies in DISLE |
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What antibody is common in 20%-50% of SLE cases?
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anti-Ro antibodies
also called anti-SS-A |
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What is the most common initial sign of systemic sclerosis?
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Raynaud's Phenomenon
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What antibodies will you see with Systemic Sclerosis?
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Anti-topisomerase Antibodies (seen in 15-40%) of cases, along with ANA in 70-90% of the time
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CREST
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Calcinosis, centromere antibody
Raynaud's phenomenon Esophageal dysmotility Sclerodactyly (tapered, claw-like fingers) Telangiectasias (multiple punctuate blood vessle findings) you will see anti-centromere antibodies in 90% of CREST patients |
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Dermatomyositis and Polymyositis
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Muscle pain and atrophy
Increased Serum Creatine Kinase Serum ANA is positive in less than 30% of cases heliotrope eyelid discoloration muscle biopsy shows lymphocytic infiltrate anti-Jo-1 |
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MIxed Connective Tissue Disorder
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See antiribonucleoprotein antibodies are positive in almost 100% of cases
signs and symptoms are similar to SLE, Systemic Sclerosis (scleroderma) and polymyositis |
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What is the most common congential immunodeficiency disorder?
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IgA deficiency-- failure of IgA B cells to mature to plasma cells
see pulmonary infections, giardiasis, and anaphylaxis if exposed to blood products containing IgA |
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thrombocytopenia, sinopulmonary infections, Eczema, decreased IgM and increased IgA and IgE
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Wisckott Aldrich Syndrome
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Thymic hypoplasia
Cerebellar Ataxia Mutation in DNA repair enzymes Increased serum alpha fetoprotein |
Ataxia Telangiectasia
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What do you think when you see recurrent strep pneumo infections?
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Congenital B cell disorders
including Bruton's Agamma, CVID, and IgA deficiency also seen with combined B and T cell deficiencies like SCID and WAS and AT |
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polymyositis
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muscle pain and weakness, shoulders are commonly involved
anti-Jo-1 and increased serum CK are seen associated with T cell mediated damage increased risk of neoplasms in 15-20% of cases, particularly lung cancer |
