Term
| Location of high endothelial venules |
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Definition
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Term
| Chronic Granulomatous Disease |
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Definition
Pathophys: NADPH oxidase deficient, less reactive oxygen species, neutrophils cannot do respiratory burst, abnormal macrophage function
Labs: negative nitroblue tetrazolium dye reduction test
Presentation: sensitive to Catalase + orgs. (S. Aureus, Nocardia, Serratia, Aspergillus, EColi, Pseudomonas Cepacia) |
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Term
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Definition
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Term
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Definition
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Term
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Definition
| Psoriasis, Ankylosing Spondylitis, IBD, Reiters |
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Term
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Definition
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Term
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Definition
| MS, Hay fever, SLE, Goodpastures |
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Term
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Definition
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Term
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Definition
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Term
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Definition
| Hashimoto's Pernicious Anemia-B12 Def |
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Term
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Definition
| steroid responsive nephrotic syndrome |
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Term
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Definition
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Term
| Antibody that crosses the placenta |
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Definition
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Term
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Definition
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Term
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Definition
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Term
| Mastcell, basophil activating antibody |
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Definition
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Term
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Definition
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Term
| Recognizes antigens w/o peptide |
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Definition
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Term
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Definition
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Term
| Primary opsonins in bacterial defense |
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
| Anti-Scl-70 (anti-DNA toporisomerase I) |
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Definition
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Term
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Definition
| primary biliary cirrhosis |
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
| Polymyositis, dermatomyositis |
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Term
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Definition
| Mixed connective tissue disease |
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Term
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Definition
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Term
| Anti-glutamate decarboxylase |
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Definition
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Term
| Bruton's agammaglobulinemia |
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Definition
Pathophys: Defect in BTK, tyrosine kinase, no B-cell differentiation.
Labs: Normal pro-B, less maturation, less number of B cells, less Igs (all)
Presentation: recurrent bacterial infections after 6 months |
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Term
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Definition
Pathophys: Defective CD40L on Th cells - no B cell class switching
Labs: High IgM, very low IgG, IgA, IgE
Presentation:severe pyogenic infections early in life |
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Term
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Definition
Pathophys: Defective isotope switching (specific Ig missing, most often IgA)
Labs: Less of Ig (IgA most likely)
Presentation: sinus and lung infections, milk allergies and diarrhea, anaphylaxis on exposure to blood with IgA |
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Term
| Common variable immunodeficiency |
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Definition
Pathophys: Defect in B-cell maturation (many causes)
Labs: normal # of B cells, less plasma cells and Ig
Presentation: sinopulmonary infections (hflu, s. pneumo), increased risk of autoimmune disease, lymphoma, can be acquired in 20s-30s |
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Term
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Definition
DiGeorge
Pathophys: 22q11 deletion, failure to develop 3rd and 4th pharyngeal pouches. Thymus and parathymus fail to develop.
Labs: less T cells, less PTH, less calcium
Presentation:tetany (hypoCa) recurrent viral/fungal infections. congenital heart/great vessel defects |
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Term
| IL-12 receptor deficiency |
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Definition
| Pathophys: decreased Th1 response Labs: less IFN gamma Presentation: disseminated mycobacterial infections |
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Term
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Definition
Pathophys:cells fail to produce IFN gamma - neutrophils can't react to chemotaxtic stimuli, hyper IgE
Labs: increased IgE
Presentation:coarse facies, noninflammed staphylococcal abcess, retained primary teeth, eczema |
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Term
| Chronic mucocutaneous candidiasis |
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Definition
Pathophys: T cell dysfxn
Presentation: candida albicans infections of skin and mucus membranes |
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Term
| Severe combined Immunodeficiency |
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Definition
Pathophys: 1. defective IL2 receptor
2. adenosine deaminase deficiency.
3. MHCII deficiency
Labs: 1. decreased T cell activation
2. increased adenine (toxic to B and T cells)
Presentation: B and T cell deficiency causes recurrent viral, bacterial, fungal and protozoal infections.
Treatment: Bone marrow transplant |
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Term
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Definition
Pathophys: Defect in DNA repair
Labs: IgA deficiency
Presentation: cerebellar defects (ataxia), spider angiomas, IgA deficiency leading to sinopulmonary infections |
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Term
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Definition
Pathophys: X linked. Progressive loss of T and B cells
Labs: more IgE and IgA, decreased IgM
Presentation: Thrombocytopenic purpura, infections, eczema |
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Term
| Leukocyte adhesion deficiency |
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Definition
Pathophys: defect in LFA-1 integrin (CD18) on phagocytes
Labs: neutrophilia
Presentation: recurrent bacterial infections, absent pus formation, delayed umbilical separation |
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Term
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Definition
Pathophys: Autosomal recessive, microtubular dysfunction --> decreased phagocytosis
Presentation: recurrent pyogenic infections by staph and strep, partial albinism, peripheral neuropathy |
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Term
| Cells involved in type IV hypersensitivity |
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Definition
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Term
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Definition
| anti-hemagglutinin IgA, IgG |
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Term
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Definition
bind to cyclophilin, which then binds to calcineurin, inhibiting transmission of IL-2
Only prevents activation of inactive cells, no function on already activated cells |
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Term
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Definition
| Inhibits Th activation by binding to a cyclophilin (different from cyclosporin), which binds to calcineurin inhibiting IL-2 formation |
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Term
| Mechanism: Mycophenolate mofetil |
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Definition
| inhibits inosine monophosphate dehydrogenase, which changes inosine monophosphate to guanosine monophosphate, thereby inhibiting B and T cells |
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Term
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Definition
| used early following transplantation Inhibits synth of inosinic acid, blocking DNA synth |
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Term
| Mechanism: Muromonab-CD3 (OKT3) |
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Definition
| binds to CD3 on T cells, blocks interaction with CD3, and blocks T cell signal transduction |
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Term
| Mechanism of immunosuppression: Corticosteroids |
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Definition
corticosteroid-receptor complex binds to NF-kappaB to prevent IL-2 transcription
Also inhibit macrophage release of IL-1 (which activates IL-2 synth) |
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Term
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Definition
| binds to mTOR, inhibits T Cell proliferation in response to IL-2 |
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Term
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Definition
| monocolonal antibody with high affinity for IL-2 receptor on activated T cells |
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Term
| Negative nitroblue tetrazolium dye reduction test |
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Definition
| Chronic granulomatous disease |
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Term
| See decreased IFN-gamma in? |
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Definition
| IL-12 deficiency and Job's syndrome |
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Term
Erythroblastosis fetalis antibody
Sx? |
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Definition
IgG
Anemia, nucleated erythrocytes, hepatomegaly, edema, extramedullary hematopoeisis |
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Term
| antibody involved in henoch-schonlein purpura |
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Definition
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Term
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Definition
| IgE-dependent basophil degranulation |
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Term
| Where complement (c1) binds |
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Definition
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Term
| Site of phagocytic cell binding |
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Definition
(CH3) Fc Receptor on phagocytic cell (macrophage, neutrophil) binds
Also site of J chain binding
[image] |
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Term
| Site of T cell positive selection |
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Definition
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Term
| Site of T cell negative selection |
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Definition
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Term
| Path of chronic rejection |
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Definition
few inflammatory cells
Fibrosis via antibody-mediated vascular damage |
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Term
| Path of hyperacute kidney rejection |
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Definition
| vascular fibrinoid necrosis, neutrophil infiltrates within arterioles, glomeruli, peritubular capillaries |
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Term
| Antibody forming dimers with J chain |
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Definition
IgA - dimer is secretory
dimer also contains secretory component |
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Term
| location of secretory component production |
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Definition
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Term
| C1 complement component deficiency |
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Definition
| associated with SLE, not immunodeficiency |
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Term
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Definition
Peptide bound to the MHCII at antibody binding site during intracellular processing.
Peptide aids transport of MHCII to phagolysosome or acidified endosome (after endosome-lysosome fusions) where antigen is. |
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Term
| cystokines involved in inflammation (increased ESR) |
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Definition
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Term
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Definition
| basoconstriction, vasoconstriction, platelet stimulation |
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Term
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Definition
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