Term
| Site of B cell localization and proliferation |
|
Definition
Follicle
located in outer cortex of LN
1 - dense and dormant
2 - pale, central germinal centers, active |
|
|
Term
| Primary follicle vs. secondary follicle? |
|
Definition
1- dense and dormant site of B cell localization
2 - pale central germinal centers, active |
|
|
Term
| Consists of closely packed lymphocytes and plasma cells and sinuses |
|
Definition
| Medulla (meduallary cords and medullary sinuses) - site on antibody production |
|
|
Term
| Houses T cells - where is this located? |
|
Definition
Paracortex
In LN, between follicles and medulla |
|
|
Term
| Area of LN that becomes greatly enlarged in extreme cellular response |
|
Definition
| Paracortex (site of T cells, viral infection) |
|
|
Term
| Are of LN underdeveloped in DiGeorge syndrome |
|
Definition
| Paracortex (lacking T cells) |
|
|
Term
| What makes up the medullary sinuses and what is its function? |
|
Definition
Contain reticular cells and macrophages
communicate with efferent lymphatics |
|
|
Term
| Where does the upper limb and lateral breast drain? |
|
Definition
|
|
Term
| Where does the stomach drain? |
|
Definition
|
|
Term
| Where does the duodenum and jejunum drain? |
|
Definition
| Superior mesenteric lymph node |
|
|
Term
| Where does the sigmoid colon drain? |
|
Definition
| colic LN --> inferior mesenteric LN |
|
|
Term
| Where does the lower rectum, anal canal above pectinate line drain to? |
|
Definition
|
|
Term
| Where does the anal canal below pectinate line drain to? |
|
Definition
|
|
Term
| Where does the testes drain to? |
|
Definition
| Superficial and deep plexsuses --> para aortic LN |
|
|
Term
| Where does the scrotum drain to? |
|
Definition
|
|
Term
| Where does the superficial thigh drain to? |
|
Definition
|
|
Term
| Where does the lateral side of the dorsum of the foot drain to? |
|
Definition
|
|
Term
| What does the Right lymphatic duct drain? |
|
Definition
| right arm and right half of head |
|
|
Term
| What does the thoracic duct drain? |
|
Definition
| everything except right arm and right half of head |
|
|
Term
| What does the superficial inguinal LN drain? |
|
Definition
Anal canal below pectinate line
scrotum (NOT TESTES)
superficial thigh |
|
|
Term
| Long vascular channels in red pulp with fenestrated barrel hoop basement membrane |
|
Definition
spleen sinusoids
macrophages nearby |
|
|
Term
| Where are T cells in the spleen? B cells? |
|
Definition
T - peri-arterial lymphatic sheath (white pulp, surrounds central arteriole) and in the red pulp of spleen
B - follicles in white pulp of spleen |
|
|
Term
| What removes encapsulated bacteria? |
|
Definition
| macrophages in the spleen |
|
|
Term
| How does splenic dysfunction causes increased susceptibility to encapsulated organisms? |
|
Definition
decreased IgM --> decreased complement activation --> decreased C3b activation --> increased susceptibility
H. flu, S. pneumo, Klebsiella |
|
|
Term
| What are Howell-jolly bodies and what should they make you think of? |
|
Definition
| nuclear remnants left because of asplenia/postsplenectomy |
|
|
Term
| What changes on a blood smear would you see post splenectomy? |
|
Definition
Howell-Jolly bodies
Target cells
thrombocytosis |
|
|
Term
| Site of T cell differentiation and maturation? |
|
Definition
|
|
Term
| Development and structure of thymus? |
|
Definition
Encapsulated
develops from epithelium of 3rd branchial pouches
Lymphocytes - mesenchymal origin
Cortex: dense with immature T cells
Corticomedullary junction: site of T cell selection
Medulla: pale with mature T cells and epithelial reticular cells and contains Hassall's corpuscles
Hassall's corpuscles - epithelial cells concentrically compressed and keratinized |
|
|
Term
| How and where are T cells selected? |
|
Definition
Thymus - selection at corticomedullary junction
1. double negative cells proliferate (4-8-; double -) in outer region of cortex, gain cell markers (4+8+; double +)
2. Positive selection (MHC restriction) - TCR recognizes self MHC (thymic stromal cells)
3. loss of aberrant 4 or 8 (depending on MHC recognition; single +)
4. Negative selection (removal of cells that react to self, dendritic cells present peptides)
Only 2% of starting population makes it! |
|
|
Term
| Components of innate immunity |
|
Definition
receptors that recognize pathogens are germline coded, fast, nonspecific response, no memory
Macrophages, PMNs, dendritic cells, NK cells, complement |
|
|
Term
| Components of Adaptive immunity |
|
Definition
Receptors that recognize pathogens undergo V(D)J recombination during lymphocyte development, slow response on first exposure, memory response is fast and robust
T cells, B cells, antibody |
|
|
Term
| What promotes development of Th1 cell and what does this cell make? |
|
Definition
INDUCES CELL MEDIATED IMMUNITY
IL-12 leads to Th1
Th1 makes TNFa and INFy and IL2
Acts to activate macrophages and CD8T
inhibited by IL-10 |
|
|
Term
| What promotes development of Th2 cell and what does this cell make? |
|
Definition
INDUCES ANTIBODY MEDIATED IMMUNITY
IL4 promotes Th2 cell generation
Th2 cells make IL4, IL5, and IL10
Stimulates B cells to make antibody (IgE>IgG)
inhibited by IFNy |
|
|
Term
| What encodes major histocompatibility complex? |
|
Definition
| Human Leukocyte Antigen genes |
|
|
Term
| MHC-I genes and cells expressed on |
|
Definition
MHC I - HLA A, B, C
expressed on all nucleated cells |
|
|
Term
| MHC II genes and cells expressed on |
|
Definition
MHC II - HLA DR, DP, DQ
expressed only on antigen presenting cells |
|
|
Term
| MHC I structure and antigen processing |
|
Definition
Antigen is loaded onto MHC I in RER (virus is digested via proteasome and then transported into RER via TAP, although most frequently bound peptide is self)
Pairs with B2 microglobin that helps with transport to cell surface
MHC I structure: 3 domain a chain paired with B2 microglobulin, peptide binding groove accommodates shorter peptides
mediates viral immunity |
|
|
Term
| MHC II structure and antigen processing |
|
Definition
Antigen is loaded following release of invariant chain in an acidified endosome
MHC II structure: a and B chain, peptide binding groove between chains, longer peptides |
|
|
Term
| What causes hyperacute organ rejection? acute? chronic? |
|
Definition
hyperacute: antibody
acute and chronic: T cells |
|
|
Term
|
Definition
reved up by IL-12, IFN-B, IFN-a
Kill when exposed to nonspecific activation signal or absence of class I MHC on target cell
Use perforin and granzymes to induce apoptosis (viral infected and tumor cells) |
|
|
Term
| What is the only lymphocyte member of the innate immune system? |
|
Definition
|
|
Term
| What cytokines enhance the activity of NK cells? |
|
Definition
|
|
Term
| Cluster of polypeptides associated with a TCR, needed for signal transduction |
|
Definition
|
|
Term
|
Definition
Dendritic cells
Macrophages
B cells
NOT neutrophils |
|
|
Term
| How do macrophages and lymphocytes interact? |
|
Definition
Lymphocytes - release IL12 stimulating macs
Macs - release IL1, TNFa stimulating lymphos |
|
|
Term
| How do superantigens work? |
|
Definition
S. pyogenes, S. aureus
cross link B region of TCR to MHC class II on APC
causes uncoordinated release of IFNy from Th1 and subsequent release of IL1, IL6, and TNFa from macrophages |
|
|
Term
| How do endotoxins/LPS work? |
|
Definition
found on G- bacteria
directly stimulates macrophages by binding to endotoxin receptor CD14
Th cells not involved |
|
|
Term
| Describe activation of Th cells |
|
Definition
1. Foreign body phagocytosed by APC
2. Antigen presented on MHC II, recognized by TCR on Th cells (signal 1)
3. B7 on APC and CD28 on Th cell interact (costimulatory signal)
4. Th cell activated! produces cytokines (IL2) |
|
|
Term
|
Definition
1. Protein presented on MHC I, recognized by TCR on Tc cell (signal 1)
2. IL-2 from Th cell activates Tc cell to kil (signal 2) |
|
|
Term
| Describe B-cell class switching |
|
Definition
- IL4/5/6 from Th2 cell (signal 1)
- CD40 receptor activation by binding CD40 ligand on Th cell (signal 2) |
|
|
Term
| Endotoxin receptor of APC |
|
Definition
CD14
activation of receptor bypasses T cells |
|
|
Term
| Role of variable part of antibody |
|
Definition
|
|
Term
| Role of Fc portion of antibody |
|
Definition
| fixes complement (IgG and IgM only) |
|
|
Term
Fc is made of?
Fab is made of? |
|
Definition
Fc - heavy chain, carboxy terminal, determines isotype
Fab - heavy and light chains, amino terminal, determines idiotype |
|
|
Term
| What holds together the two heavy chains? |
|
Definition
| interchain disulfide bond |
|
|
Term
| Describe the Fc portion of antibodies |
|
Definition
Constant
Complement binding (IgM, IgG)
carbohydrate side chains
determines isotype |
|
|
Term
| How is antibody diversity generated? |
|
Definition
1. random recombo of VJ (light) and VDJ (heavy) chain genes
2. random combo of heavy and light chains
3. Somatic hypermutation (following antigenic stimulation)
4. Addition of nucleotides to DNA during recombination by terminal deoxynucleotidyl transferase |
|
|
Term
| Which side is the amino terminal of the antibody? |
|
Definition
|
|
Term
|
Definition
1. opsonization (promotes phagocytosis)
2. neutralization (prevents bacterial adherence)
3. Complement activation (enhance opsonization and lysis) |
|
|
Term
| Mature B cells express what antibodies? |
|
Definition
|
|
Term
| Differentiation of mature B cells leads to what? |
|
Definition
| Isotype switching (alternate mRNA splicing, mediated by cytokines and CD40 ligand) into plasma cells that secrete IgA, IgE, or IgG |
|
|
Term
|
Definition
|
|
Term
| Antibody that fixes complement, crosses placenta, opsonizes bacteria, neutralizes bacterial toxins and viruses |
|
Definition
|
|
Term
| Antibody that prevents attachment of bacteria and viruses to mucous membranes |
|
Definition
|
|
Term
| Where is IgA found? what is its structure? |
|
Definition
In secretions
monomer or dimer |
|
|
Term
| Where does IgA's secretory component come from? |
|
Definition
| from epithelial cells before secretion |
|
|
Term
| Which two antibodies fix complement? |
|
Definition
|
|
Term
| Antibody that serves as antigen receptor on B cells |
|
Definition
|
|
Term
|
Definition
monomer on B cells
pentamer |
|
|
Term
| Found on the surface of many B cells and in serum; unclear function |
|
Definition
|
|
Term
| Mediates type I (immediate) hypersensitivity |
|
Definition
|
|
Term
| How does IgE mediate type I hypersensitivity? |
|
Definition
Binds to mast cells and basophils
releases mediators when exposed to allergen |
|
|
Term
| What antibody mediates immunity to worms? |
|
Definition
| IgE, activates eosinophils |
|
|
Term
| What antibody is in lowest concentration in serum? |
|
Definition
|
|
Term
| Ig allele that differs among members of same species |
|
Definition
allotype (polymorphism)
determined by heavy of light chain |
|
|
Term
| Ig region common to single class of Ig |
|
Definition
Isotype (IgG, IgA, etc)
determined by heavy chain |
|
|
Term
| Ig epitope determined by antigen binding sites |
|
Definition
|
|
Term
| What is a thymus independent antigen? examples? |
|
Definition
Antigen without peptide component, not presented by MHC to T cells
Stimulate release of IgM, no immunologic memory
LPS form cell envelope of G-, polysaccharide capsular antigen |
|
|
Term
| What is a thymus dependent antigen? examples? |
|
Definition
Antigen with protein component, presented
Induces class switching and immunologic memory via direct contact of B cells with Th cells (CD40-CD40 ligand interaction), release of IL4/5/6
conjugated H. Flu vaccine |
|
|
Term
Secreted by macrophages
causes acute inflammation
induces chemokine production to recruit leukocytes
activates endothelium to express adhesion molecules
endogenous pyrogen |
|
Definition
|
|
Term
Secreted by macrophages
mediates septic shock
causes leukocyte recruitment, vascular leak |
|
Definition
|
|
Term
Secreted by macrophages
major chemotactic factor for neutrophils |
|
Definition
|
|
Term
Secreted by Th cells and macrophages
stimulates production of acute-phase reactants and immunoglobulin |
|
Definition
|
|
Term
Secreted by Th1 cells
stimulates macrophages |
|
Definition
|
|
Term
Secreted by Th cells
stimulates growth of helper and cytotoxic T cells |
|
Definition
|
|
Term
Secreted by Th2 cells
promotes growth of B cells
Enhances class switching to IgE and IgG |
|
Definition
|
|
Term
Secreted by activated T cells
supports the growth and differentiation of bone marrow stem cells
function similar to GM-CSF |
|
Definition
|
|
Term
Secreted by Th2 cells
promotes differentiation of B cells
enhances class switching to IgA
stimulates production and activation of eosinophils |
|
Definition
|
|
Term
Secreted by regulatory T cells
Inhibits actions of activated T cells |
|
Definition
|
|
Term
Secreted by B cells and macrophages
Activates NK and Th1 cells |
|
Definition
|
|
Term
| Cell surface proteins on Helper T cells |
|
Definition
CD4
TCR
CD3
CD28 (interacts with B7 on APCs)
CD40L (signal for B cell class switching) |
|
|
Term
| Cell surface proteins on cytotoxic T cells |
|
Definition
|
|
Term
| Cell surface proteins on B cells |
|
Definition
IgM
B7 (interacts with Th CD28, activates Th cell)
CD19, CD20, CD21 (EBV receptor)
CD40 (interacts with Th CD40L, class switching stimulated)
MHC II |
|
|
Term
| Cell surface proteins on macrophages |
|
Definition
MHC II
B7 (interacts with Th CD28)
CD40
CD14 (endotoxin receptor)
Receptors for Fc and C3b |
|
|
Term
| Cell surface proteins on NK cells |
|
Definition
Receptors for MHC I
CD16 (binds Fc of IgG)
CD56 |
|
|
Term
| Cell surface proteins on all cells except mature RBCs |
|
Definition
|
|
Term
| Complement that aids in clearance of immune complexes |
|
Definition
|
|
Term
| What helps prevent complement activation on self-cells? |
|
Definition
Decay accelerating factor
C1 esterase inhibitor |
|
|
Term
| What activates classical complement pathway? |
|
Definition
| IgM or IgG on the surface of microbes |
|
|
Term
| What activates the alternative complement pathway? |
|
Definition
| Molecules on the surface of microbes (ie. endotoxin) |
|
|
Term
| What complement components are involved in anaphylaxis? |
|
Definition
|
|
Term
| What complement component is involved in neutrophil chemotaxis? |
|
Definition
| C5a (also involved in anaphylaxis) |
|
|
Term
| What causes hereditary angioedema? |
|
Definition
| deficiency in C1 esterase inhibitor |
|
|
Term
| What complement deficiency leads to increased susceptibility to type III hypersensitivity? |
|
Definition
C3 deficiency
C3 involved in clearance on immune complexes
also makes you susceptible to recurrent pyogenic sinus and respiratory tract infections |
|
|
Term
|
Definition
| leads to Neisseria bacteremia (no MACs) |
|
|
Term
|
Definition
DAF = GPI-anchored enzyme
deficiency leads to complement mediated lysis of RBCs and paroxysmal nocturnal hemoglobinuria |
|
|
Term
|
Definition
Place uninfected cells in antiviral state.
Induce production of ribonuclease that inhibits viral protein synthesis by degrading viral mRNA (but not host mRNA) |
|
|
Term
| Roles of a, b, and y interferon |
|
Definition
a and B - inhibit viral protein synthesis
y - increase MHC I and II expression and antigen presentation in all cells
all - activate NK cells to kill virus infected cells |
|
|
Term
| How long is passive immunity good for? |
|
Definition
| Ab T1/2 = 3 weeks (ie IgA in breast milk) |
|
|
Term
| What infections can be stopped with passive immunity? |
|
Definition
Tetanus toxin
Botulinum toxin
HBV
Rabies virus |
|
|
Term
| What bacteria are capable of antigen variation? |
|
Definition
1. Salmonella (2 flagellar variants)
2. Borrelia (relapsing fever)
3. N. Gonorrhoeae (pilus protein)
variation mechanisms: DNA rearrangement, RNA segment rearrangment |
|
|
Term
| What parasite is capable of antigen variation? |
|
Definition
| Trypanasomes (programmed rearrangement) |
|
|
Term
|
Definition
Self reactive T cells become nonreactive without costimulatory molecule
B cells - also occurs, but less complete than in T cells |
|
|
Term
|
Definition
Giant cells
epithelioid cells
fibroblasts
lymphocytes |
|
|
Term
| Name 8 granulomatous diseases |
|
Definition
1. TB
2. Leprosy
3. Cat scratch fever
4. Syphilis
5. Fungal infections (histo)
6. Sarcoidosis
7. Crohn's
8. Berylliosis |
|
|
Term
| How does granulomatous disease develop? |
|
Definition
APC cell presents antigen
Th cell activated (promoted by self IL2 release)
Releases IFN-y
stimulates Monocyte, macrophage, epithelioid cell and giant cell development
granuloma = epitheloid cell+giant cell+fibroblast+lymphocytes |
|
|
Term
| What is a Type I hypersensitivity |
|
Definition
Anaphylactic and atopic
due to preformed antibody (IgE) on presensitized mast cells and basophils, triggers release of vasoactive amines that act at postcapillary venules |
|
|
Term
| A scratch test or radioimmunosorbent assay test what? |
|
Definition
|
|
Term
| What is a type II hypersensitivity? |
|
Definition
Antibody mediated - IgM/IgG bind to fixed antigen on enemy cell, leads to lysis (by complement) or phagocytosis
cytotoxic
test: direct and indirect coombs |
|
|
Term
| Direct and indirect Coombs test what? |
|
Definition
|
|
Term
| What 3 mechanisms does a type II hypersensitivity work through? |
|
Definition
1. activates complement or opsonize cell
2. Ab recruit neutrophils and macrophages that incite tissue damage
3. bind to normal cellular receptors and interfere with function |
|
|
Term
| How does type III hypersensitivity happen? |
|
Definition
| antigen+antibody (IgG) complexes activate complement, attracts neutrophils which release lysosomal enzymes |
|
|
Term
|
Definition
Type III hypersensitivity (immune complex disease)
-ab to foreign proteins are produced (5 days), immune complexes form, deposited in membranes, fix complement, leads to tissue damage
most often caused by drugs - fever, urticaria, arthralgias, proteinuria, lymphadenopathy 5-10d post antigen exposure |
|
|
Term
|
Definition
local subacute Type III reaction
intradermal injection of Ag induces Ab = form complexes in skin
edema, necrosis, activation of complement |
|
|
Term
| Immunofluorescent staining tests for this hypersensitivity |
|
Definition
|
|
Term
|
Definition
sensitized T cells encounter antigen
release lymphokines
activate macrophages
cell mediated, not transferable by serum
Transplant rejections, TB skin test, Touching (contact dermatitis)
patch test |
|
|
Term
| Name the type of hypersensitivity: anaphylaxis |
|
Definition
|
|
Term
| Name the type of hypersensitivity: allergic rhinitis |
|
Definition
|
|
Term
| Name the type of hypersensitivity: contact dermatitis |
|
Definition
|
|
Term
| Name the type of hypersensitivity: hemolytic anemia |
|
Definition
|
|
Term
| Name the type of hypersensitivity: pernicious anemia |
|
Definition
| II (IF on parietal cells) |
|
|
Term
| Name the type of hypersensitivity: idiopathic thrombocytopenic purpura |
|
Definition
| II (gpIIb/IIIa on platelets) |
|
|
Term
| Name the type of hypersensitivity: erythroblastic fetalis |
|
Definition
| II (antigens on newborns erythrocytes) |
|
|
Term
| Name the type of hypersensitivity: rheumatic fever |
|
Definition
| II (M proteins on Group B strep and then myocardium) |
|
|
Term
| Name the type of hypersensitivity: Goodpastures syndrome |
|
Definition
|
|
Term
| Name the type of hypersensitivity: Bullous pemphigoid |
|
Definition
|
|
Term
| Name the type of hypersensitivity: pemphigus vulgaris |
|
Definition
|
|
Term
| Name the type of hypersensitivity: Graves disease |
|
Definition
| II (thyroid TSH receptor) |
|
|
Term
| Name the type of hypersensitivity: myasthenia gravis |
|
Definition
| II (Ach receptors post synaptic) |
|
|
Term
| Name the type of hypersensitivity: SLE |
|
Definition
|
|
Term
| Name the type of hypersensitivity: RA |
|
Definition
|
|
Term
| Name the type of hypersensitivity: Polyarteritis nodosum |
|
Definition
| III (HBV antigen-ab complex leads to systemic vasculitis) |
|
|
Term
| Name the type of hypersensitivity: poststrep glomerulonephritis |
|
Definition
|
|
Term
| Name the type of hypersensitivity: serum sickness |
|
Definition
|
|
Term
| Name the type of hypersensitivity: swelling/inflammation following tetanus vaccine |
|
Definition
|
|
Term
| Name the type of hypersensitivity: farmer's lung |
|
Definition
| III (hypersensitivity pneumonitis) |
|
|
Term
| Name the type of hypersensitivity: hypersensitivity pneumonitis |
|
Definition
|
|
Term
| Name the type of hypersensitivity: type I DM |
|
Definition
|
|
Term
| Name the type of hypersensitivity: MS |
|
Definition
|
|
Term
| Name the type of hypersensitivity: Guillain-Barre syndrome |
|
Definition
|
|
Term
| Name the type of hypersensitivity: Hashimotos syndrome |
|
Definition
|
|
Term
| Name the type of hypersensitivity: GVHD |
|
Definition
|
|
Term
| Name the type of hypersensitivity: PPD |
|
Definition
|
|
Term
| Where is the problem in SCID? |
|
Definition
1. ADA deficiency
Blocks Lymphoid SC development into pro-B and pro-T cells
2. defective IL-2 receptor, no development of T cells beyond pro-T cells
3. MHC II deficiency - no activation of CD4+ T cells |
|
|
Term
| What is the problem in Burton's agammaglobulinemia? |
|
Definition
| No generation of immature B cells from Pre-B cell |
|
|
Term
| What is the problem in Di george syndrome? |
|
Definition
| No development of Immature T cells |
|
|
Term
| What is the problem in CVID? |
|
Definition
| No development of immature B cells (no IgM or IgG B cells, etc.) |
|
|
Term
| What is the problem in hyper-IgM syndrome? |
|
Definition
No class switching
only IgM producing B cells |
|
|
Term
| What is the inheritance of Burton's agammaglobulinemia? Gene? |
|
Definition
|
|
Term
| Associated with low levels of all classes of Ig but normal number of pro-B cells in marrow |
|
Definition
| Burtons agammaglobulinemia |
|
|
Term
| Immunologic condition that presents with tetany. Why? |
|
Definition
DiGeorge
hypocalcemia due to missing parathyroid glands |
|
|
Term
| Describe problems seen in Di George syndrome |
|
Definition
hypocalcemia (tetany)
athymic (recurrent viral and fungal infections)
Congenital heart defects
Congenital great vessel defects
22q11 deletion |
|
|
Term
| Most common form of SCID? what are the other forms? |
|
Definition
Defective IL-2 receptors (X linked)
others: failure to synthesize MHC II antigens, adenosine deaminase deficiency
no rejection fo allografts |
|
|
Term
| Presents with disseminated mycobacterial infections due to decreased Th1 response |
|
Definition
| IL-12 Receptor deficiency of T cells |
|
|
Term
| Defect in CD40L on CD4 T cells |
|
Definition
HyperIgM syndrome
no class switching
high IgM, low IgG/A/E |
|
|
Term
| Inheritance of Wiskott-Aldrich syndrome? Triad of symptoms? |
|
Definition
X-linked defect in ability to mount IgM response to capsular polysaccharides
Recurrent pyogenic infection
Thrombocytopenic purpura
Eczema |
|
|
Term
| Recurrent pyogenic infections, thrombocytopenic purpura, eczema |
|
Definition
| Wiskott-aldrich (X-linked defect in ability to mount IgM response to encapsulated bacteria) |
|
|
Term
| Failure of IFN-y production by helper T cells |
|
Definition
Job's syndrome
neutrophils fail to respond to chemotactic stimuli
Coarse facies, Abscesses (cold, staph), Teeth retained, increased IgE, derm problem (eczema) |
|
|
Term
| Coarse facies, cold Staph abscesses, retained primary teeth, increased IgE, dermatologic problems |
|
Definition
| Jobs syndrome (failure of IFN-y production by Th cells, no neutrophil response) |
|
|
Term
| Recurrent bacterial infections, absent pus formation, neutrophilia, delayed separation of umbilicus |
|
Definition
Leukocyte adhesion deficiency
defect in LFA-1 integrin (CD18) proteins on phagocytes |
|
|
Term
| Inheritance of Chediak higashi syndrome? defect? |
|
Definition
| AR, defect in microtubule function and lysosomal emptying |
|
|
Term
| Presents with recurrent pyogenic infections by staph and strep, partial albinism, peripheral neuropathy |
|
Definition
|
|
Term
| Lack of NADPH oxidase activity |
|
Definition
Chronic granulomatous disease
defect in microbiocidal activity on neutrophils |
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Term
| How do you confirm diagnosis of CGD? |
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Definition
| negative nitroblue tetrazolium dye reduction test |
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Term
| Presents with skin and mucous membrane Candida infections? caused by what dysfunction? |
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Definition
Chronic mucocutaneous candidiasis
T cell dysfunction |
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Term
| Most common selective Ig deficiency? how does it present? |
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Definition
IgA
sinus, lung infections, milk allergies, diarrhea, anaphylaxis with exposure to blood products containing IgA |
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Term
| Defect in DNA repair enzymes and associated IgA deficiency |
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Definition
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Term
| Cerebellar problems, spider angiomas, IgA deficiency |
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Definition
| Ataxia telangiectasia (defect in DNA repair enzyme) |
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Term
| Normal number of circulating B cells but decreased plasma cells and Ig |
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Definition
CVID
can be acquired in 20-30s
increased risk of autoimmune disease and lymphoma |
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Term
| CVID has an increased risk of what associated with it? |
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Definition
autoimmune disease
lymphoma |
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Term
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Definition
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Term
| Disease association: Anti-dsDNA |
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Definition
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Term
| Disease association: Anti-smith |
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Definition
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Term
| Disease association: Antihistone |
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Definition
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Term
| Disease association: Anti-IgG |
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Definition
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Term
| Disease association: Anti-centromere |
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Definition
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Term
| Disease association: Anti-Scl-70 |
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Definition
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Term
| Disease association: Anti-mitochondrial |
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Definition
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Term
| Disease association: Anti-gliadin |
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Definition
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Term
| Disease association: Anti-endomysial |
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Definition
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Term
| Disease association: Anti-basement membrane |
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Definition
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Term
| Disease association: Anti-desmoglein |
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Definition
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Term
| Disease association: Anti-microsomal |
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Definition
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Term
| Disease association: Anti-thyroglobulin |
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Definition
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Term
| Disease association: Anti-Jo-1 |
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Definition
polymyositis
dermatomyositis |
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Term
| Disease association: Anti-SS-A |
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Definition
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Term
| Disease association: Anti-Ro |
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Definition
Anti-SS-A
Sjorgen's syndrome |
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Term
| Disease association: Anti-SS-B |
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Definition
Anti-La
Sjorgen's syndrome |
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Term
| Disease association: Anti-La |
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Definition
Anti-SS-B
Sjorgen's syndrome |
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Term
| Disease association: Anti-U1 RNP |
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Definition
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Term
| Disease association: Anti-smooth muscle |
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Definition
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Term
| Disease association: Anti-glutamate decarboxylase |
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Definition
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Term
| Disease association: c-ANCA |
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Definition
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Term
| Disease association: p-ANCA |
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Definition
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Term
| Disease association: HLA A3 |
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Definition
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Term
| Disease association: HLA B27 |
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Definition
| Psoriasis, Ankylosing spondylitis, IBD, Reiter's syndrome (PAIR) |
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Term
| Disease association: HLA B8 |
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Definition
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Term
| Disease association: HLA DR2 |
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Definition
| MS, hay fever, SLE, goodpastures |
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Term
| Disease association: HLA DR3 |
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Definition
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Term
| Disease association: HLA DR4 |
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Definition
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Term
| Disease association: HLA DR5 |
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Definition
Pernicious anemia
Hashimoto's thyroiditis |
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Term
| Disease association: HLA DR7 |
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Definition
| Steroid responsive nephrotic syndrome |
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Term
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Definition
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Term
| Graft from identical twin or clone |
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Definition
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Term
| Graft from nonidentical individual of same species |
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Definition
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Term
| Graft from different species |
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Definition
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Term
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Definition
Ab mediated, preformed antidonor antibodies in transplant recipient
minutes post transplantation |
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Term
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Definition
cell mediated
cytotoxic T cells react against foreign MHCs
weeks post transplant
reversible with immunosuppressants (cyclosporine, OKT3) |
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Term
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Definition
T cell and Ab mediated vascular damage (obliterative vascular fibrosis)
months to years post transplant
irreversible
Class I-MHC (nonself) is perceived by CTLs as class I-MHC(self) presenting nonself antigen |
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Term
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Definition
grafted immunocompetent T cells proliferate in irradiated ICH
reject cells with foreign proteins
results in severe organ dysfunction
maculopapular rash, jaundice, hepatosplenomegaly, diarrhea |
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