Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
38 Cards in this Set
- Front
- Back
Hyper-IgM syndrome
|
AID deficiency or CD40L deficiency
-inability to class-switch -defective opsonization -defective macrophage/DC function due to CD40L absence -increased infection by bacteria |
|
Factor H/Factor I/MCP
deficiency |
-hemolytic uremic syndrome
-Absent Factor I means constitutive C3bBb, constitutive cleavage of C3, constitutive production of C3a-->inflammation -Infections (C3 deficiency-all used up) -macular degeneration (due to inflammation) |
|
C1-INH deficiency
|
-hereditary angioedema (chronic inflammation/perfusion)
|
|
Crohn's disease
|
chronic bloody diarrhea/infection
-could be due to NOD deficiency, inability to activate defensin secretion in host cells in response to internalized pathogens |
|
Septic shock
|
sepsis causes systemic TNF-alpha secretion
-hemodilation, permeability -systemic platelet activation = inability to clot anywhere -induced by LPS |
|
Chronic granulomatous disease
|
mutation in NADPH oxidase
-inability to make ROS in macros/neutros -results in chronic bacterial/fungal infections -antigen presented normally, T cell IFN-gamma released normally. No kill. -Proliferating T cells form a granuloma around APC |
|
Chediak Higashi
|
defective phagosome/lysosome fusion
-normal ROS production -No APC presentation -albinism and death by 10 years |
|
Tuberculosis
|
impairs macro-kill by blocking phagosome/lysosome fusion
|
|
Allergy
|
IgE binds to Fc receptors in mast cells--degranulate
-granules: tryptase, chymase, carboxypeptidase, histamine, heparin (toxic to parasites) -cytokines also released (TNF, IL-4, IL-13, IL-3, IL-5) for T cells, macros, and eosinophils -specific allergies result from high titer of specific antibody |
|
Grave's Disease
|
hyperthyroidism
-autoantibody to TSH-R (agonistic) -palpitation, nervousness, fatigue, weight loss -eventually destroys thyroid cells-->no more TH |
|
Myasthenia Gravis
|
Antibodies toward ACh-receptor
-antagonistic -fatigue, muscle weakness -thymic hyperplasia (auto-antibodies target thymus) -ACh-R antibodies can be transferred maternal-fetally |
|
ICD
|
Immune complex disease
-immune complexes form in vessels, glomeruli -can develop from over-abundant antigen -activates complement, mast cell, inflammation, occlusion |
|
Goodpasture's syndrome
|
Glomerulonephritis
-Auto-antibodies directed to glomerular basement membrane prevent podocyte filtration |
|
Multiple Schlerosis
|
Auto-antibodies for myelin sheath
-mediated by T-cells for CNS antigens -induced by blood brain barrier permeable to leukocytes/blood proteins -mast cells, complement, antibodies, cytokines |
|
Type 1 DM
|
T-cells specific for beta-cells
-must eliminate 90% of beta-cells (non mitotic) to develop diabetes -NOT through antibodies |
|
Contact sensitivity
|
T cells specific for skin pathogens
-delayed sensitivity reactions -requires 2 exposures (memory T cells) -DC's present langerhans to naive capillary T cells |
|
AIRE mutation
|
Necessary for tissue-specific random expression in thymic epithelium.
-mutations result in autoimmunity |
|
Rheumatic fever
|
After infection w/ Strep pyogenes
-heart, joints, skin, brain -X-reactive antibodies between microbe and host (heart valves) -T cell activation, B cell maturation-->auto immunity -After strep is cleared, usually T-cells inactivate, self-limiting |
|
Tuberculoid leprosy
|
Th1 effector
-IL-2, IFN-gamma, TNF -low [microbe], normal Ig levels, T cells responsive |
|
Lepromatous leprosy
|
Th2 effector
-IL-4, IL-5, IL-10 -high [microbe], hypergammaglobulinemia, low T-cell response |
|
Direct allorecognition
|
graft antigens are presented by donor MHC
-recognized as a foreign antigen -presentor is donor APC |
|
Indirect allorecognition
|
graft minor histocompatibility antigens (non-MHC) are presented by recipient APCs
|
|
Hyperacute rejection
|
within 24 hours due to ABO blood mismatch
-mediated by natural antibodies |
|
Acute rejection
|
T-cell mediated
-CTL and macro cytotoxic killing -Delayed effect w/ antibody against donor endothelium |
|
Chronic rejection
|
Scarring due to repeated acute rejections
-chronic inflammation -platelet deposition (arteriosclerosis), ischemia |
|
Cross-matching
|
mix transplant recipient serum w/ donor blood
-agglutination/lysis means preformed antibodies to donor blood are present. |
|
Graft-vs-host disease
|
transplanted T-cells through bone marrow
-allogenic toward host -must first destroy donor T cells w/ antibodies (bone marrow will be re-educated by thymus) |
|
Microchimerism
|
Graft not killed
-graft tissue detectable in host skin and lymph nodes -host cells detected in graft tissue -possible autoimmunity |
|
Xenotransplantation
|
hyperacute rejection of galactosyl by natural antibodies.
|
|
Immune complex disease
|
Autoimmunity, chronic infections/antibiotics
-occurs in zone of equivalence antigen:antibody -complexes deposit near antigen -Fc's recruit neutrophils and macrophages -occludes organs w/ abundant capillaries |
|
Lupus Erythematosis
|
Autoimmune
-antigen is RBC nucleus (released in RBC differentiation) -Rash due to immune complex in skin capillary -requires auto-reactive T and B cell -lupus flair w/ cell damage -complexes not cleared unless w/ immunosuppression |
|
Wheal and Flare
|
vasodilation at lesion, bump in middle
-due to mast cell response to intradermal antigen -immediate allergic-type reaction |
|
Asthma
|
Exercise-induced allergic response
|
|
Dermatographia
|
no specific antigen.
-hypersensitive mast cells |
|
Factor I deficiency
|
Poor innate immune response (macrophage doesn't phagocytose)
-eventually reduces C3 due to constant metabolism |
|
X-linked agammaglobulinemia
|
Btk tyrosine kinase mutation.
-NO B CELLS -no serum immunoglobulins -can be in female if x-inactivated |
|
MHC II deficiency
|
Always fatal.
-no thymic selection of T cells -bone marrow transplant is useless |
|
LFA-1, I-CAM mutation
|
T cell-B cell, monocyte adhesion compromised.
|