Reading...
Play button
Play button
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;
298 Cards in this Set
- Front
- Back
|
Complement pathway
|
217-218
|
|
|
Racoon eyes & purple patches on PIP/MCP
|
Dermatomyositis, polyomyositis
|
|
|
Classic pathway
|
Ig -> C1q -> C1r -> C1s -> C4 & C2 -> C4b2a -> C5 & C3 -> C5b -> MAC
|
|
|
Lab shows
|
Inc Cr kinase
|
|
|
C5a, C3a fn? 3
|
Anaphylaxis! Mast cell histamine release; PMN chemotaxis
|
|
|
MCTD presents like SLE, PM, and SS except
|
renal dz is uncommon
|
|
|
C3b fn? 3
|
Receptor CR1; membrane binding opsinin; viral aggregation
|
|
|
Most common congenital immunodef
|
IgA
|
|
|
Alternative pathway?
|
Zymosan, IgG, IgA, endotoxin -> C3 activation
|
|
|
In polarized light, amyloid has
|
apple green birefrigence
|
|
|
Lecthin pathway?
|
MBC -> MASP -> C4 & C2 activation
|
|
|
On H&E, amyoid is
|
eosinophilic
|
|
|
C1q promoted by? 2
|
Kallikrein, plasmin
|
|
|
3 proteins that can form amyloid
|
albumin, calcitonin, light chains
|
|
|
Fluid phase protein C1NH inh? 3
|
Kallikrein, plasmin, C1r->C1s
|
|
|
Why does Down's syndrome present with early Alzheimer's?
|
Amyloid precursor for Ab coded by chr 21
|
|
|
DAF (surface protein) inh? 1
|
C4b2a
|
|
|
Primarily amyloidosis (AL) is associated with
|
Multiple myeloma
|
|
|
Fluid phase proteins Factor H, I, C4BP inh?
|
C4b, C3b
|
|
|
Secondary amyloidosis (AA) is associated with
|
chronic infl
|
|
|
Neisseria infections occur with which mutations?
|
Lecthin or alt paths
|
|
|
Auto R disorde inv AA amyloid
|
familial Mediterrean fever
|
|
|
Pyogenic infections occur with which mutations?
|
MBC, C3
|
|
|
Detected by? 2
|
Immunoelectrophoresis, tissue Bx
|
|
|
Paroxysmal nocturnal hemaglobinuria is a defect in? Angioedema?
|
DAF
C1NH |
|
|
Immunoglobins
|
58
|
|
|
Names all the types. 5
|
IgM, IgG, IgA, IgE, IgD
|
|
|
Multimeric Ig? 2
|
IgM (5), IgA (2)
|
|
|
Fetal Ig?
|
IgM
|
|
|
1st in immune response?
|
IgM
|
|
|
Highest Ig concentration in blood
|
IgG
|
|
|
Fixes complement? 2
|
IgM > IgG
|
|
|
Highest Ig concentration in secretions
|
IgA
|
|
|
Allergy Ig
|
IgE
|
|
|
Expressed on B cell surface w/ Ag receptor facing out? 2
|
IgD, IgM
|
|
|
Contains J components? 2
|
IgM, IgA
|
|
|
2nd in immune response?
|
IgG
|
|
|
Better at agglutinating?
|
IgM
|
|
|
Better at precipitating?
|
IgG
|
|
|
Cross placenta
|
IgG
|
|
|
Secretory component?
|
IgA
|
|
|
Binds to mast cells, eosinophils
|
IgE
|
|
|
Involved in parasitic infections?
|
IgE
|
|
|
B cell path
|
104
|
|
|
Steps? 6
|
Lymphoid precursor -> Pro-B -> preB -> imm B cell -> mature B -> memory, plasma cell
|
|
|
First rearrangement in?
|
Lymphoid -> pro-B
|
|
|
Purpose of VDJ rearrangement? 2
|
pro -> pre-B; brings closer to m
|
|
|
First receptor made?
|
CD79 a, b w/ surrogate light chain
|
|
|
Light chain rearrangement genes? 2
|
kl
|
|
|
Testing of B cells? 2
|
Immature B react to self -> delete or receptor editing; BCR react w/ Ag -> anergy
|
|
|
Role of T cells? 2
|
Somatic hypermutation, class switch
|
|
|
T cell path
|
136-137
|
|
|
Steps? 8
|
Lymphoid precursor -> thymus -> double neg -> pre-TCR -> double + -> + selection -> neg selection -> single +
|
|
|
Pre-TCR made by?
|
b rear for CD3
|
|
|
Double + cell contain? 2
|
CD4, CD8
|
|
|
Positive selection? 2
|
Via epithelial cells; see if recognize self MHC
|
|
|
Negative selection? 2
|
Via macrophages, DC, epi cells; makes sure not recognize self
|
|
|
Type 1 HSR
|
233
|
|
|
Pathway? 9
|
Allergen -> IgE -> FceR1 -> crosslinking -> signal transduction -> cAMP incr & dec -> Ca release -> preformed granules + activate PLC
|
|
|
PLC role?
|
Cleave out arachodonic acid from membrane
|
|
|
Arachodonic acid forms? 3
|
PG, TX, LT
|
|
|
Which granules are formed?
|
Secondary
|
|
|
What are primary granules?
|
Preformed granules
|
|
|
What do they contain? 3
|
Vasoactive amines: histamine, 5HT, protease
|
|
|
Tests for HSR? 3
|
Skin test, RIST-IgE, RAST-allergen
|
|
|
Allergy shot blocks which step?
|
Allergen -> IgE
|
|
|
Mab blocks which step?
|
IgE -> FceR1
|
|
|
Cortisone blocks which steps? 2
|
Signal transduction, vasoactive amine release
|
|
|
Cromylyn Na blocks?
|
Stabilizes membrane so no incr in Ca
|
|
|
Epinephrine blocks?
|
Signal transduction
|
|
|
Type 2 HSR
|
244
|
|
|
Mechanisms? 3
|
IgM > IgG -> activate whole complement sequence -> cell lysis
IgG & C3b act on phagocyte-R -> cytotoxic fx Dz causing ant/agonistic Ab act on cell surface R |
|
|
Cells involved? 3
|
Neutrophil, NK, CTL
|
|
|
Treated with? 3
|
Steroids with azathioprine or MTX
|
|
|
Example?
|
Goodpasture's syndrome
|
|
|
Type 3 HSR
|
244
|
|
|
Mechanism? 3 steps
|
IgG-soluble Ag complex activate complment cascade -> neutrophils ingest complex but also release degradative enzymes -> tissue damage
|
|
|
Cells involved? 2
|
Neurophils, mast cells
|
|
|
Examples? 4
|
Serum sickness, Farmer's lung, Arthrus reaction, SLE glomerulitis
|
|
|
Treated with? 3
|
Steroids, azathioprine, cyclophosphamide
|
|
|
4 T's of Type IV HSR?
|
T lymphocytes, Transplant rejections, TB skin tests, touching (contact dermatitis)
|
|
|
Rejections
|
295
|
|
|
Hyperacute mediated by?
|
Humoral
|
|
|
Cells involved? 1
|
Neutrophils
|
|
|
Ab are?
|
preformed
|
|
|
Acute medated by?
|
Humoral & cell-mediated
|
|
|
Treated with? 2
|
Steroid, OKT3
|
|
|
Pathology? 1
|
T cell in interstitium
|
|
|
Signs of chronic rejection? 3
|
Interstitial fibrosis, sclerosis of vessels, tubules and glomeruli disappear
|
|
|
Immune cells
|
|
|
|
Markers on B cell? 3
|
CD40, B7, CD29
|
|
|
CTL markers? 5
|
CD29, CD8, CD28, CTLA-4, fasL
|
|
|
CTL activated by?
|
DC
|
|
|
Dendritic cell markers?
|
B7: APC costimulatory signal interact w/ CD28 on TH
|
|
|
Best APC?
|
Dendritic cells
|
|
|
Other APC?
|
B, monocytes
|
|
|
DC licensed by?
|
TH
|
|
|
Eosinophil granule contain?
|
MBP
|
|
|
M cell function?
|
Pinocytose GI content and pass to APC
|
|
|
Mast cells stimulated by? 3
|
C3a, C5a, IgE
|
|
|
Release? 4
|
Hist, LT, PG, 5HT
|
|
|
Monocytes are part of what immmunities? 2
|
Innate; initiate adaptive
|
|
|
Stimulated by?
|
IFN-g
|
|
|
Release? 7
|
IL-1, IL-6, IL-8, IL-12, NO, ROS, ONOO, TNF-a
|
|
|
Receptors? 4
|
B7 = APC R for presenting to TH
Mac-1=CD3-R, TLR (respond to LPS) CD14 = T-indepedent stimulation via LPS |
|
|
Neutrophil half life?
|
1-2d
|
|
|
Marker?
|
CR3
|
|
|
What is it's chemokine?
|
C5a
|
|
|
Granules contain? 4
|
Lactoferrin, lysozyme, cationic proteins, defensin
|
|
|
NK stimulated by? 3
|
IL-12, IFN-b, IFN-a
|
|
|
Secrete?
|
IFN-g
|
|
|
Most important protein on surface? 2
|
FasL; kills non-MHC I cells with direct contact
|
|
|
TH1 cell markers?
|
CD29, CD4, CD28, CD2
|
|
|
TH1 class restricted to?
|
MHC I
|
|
|
Release? 3
|
IL-2: promote proliferation
IFN-g: Inh TH2, promote macrophages TNF-b |
|
|
Function? 2
|
Licence DC, help CTL (co-stimulation, signal 2)
|
|
|
TH2 cell markers? 5
|
CD29, CD4, CD2
CD28 interact w/ B7 on APC -> activates TH2 (signal 2) CD40L interact w/ CD40 on B cell -> class switching (signal 2) |
|
|
TH2 class restricted to?
|
MHC II
|
|
|
Release? 6
|
IL-13
IL-10: inh TH1 prolif IL-4, 5, 6 for class switching (signal 1) IL-4 -> IgE, IgG IL-5 -> IgA |
|
|
TH3 cell markers?
|
CD29, CD28
|
|
|
Release? 3
|
IL-4, IL-10, TGF-b
|
|
|
Deficiency is linked to?
|
Chron's dz
|
|
|
T17 markers? 3
|
CD29, CD28, CD4
|
|
|
Produces? 2
|
IL-17, IL-21
|
|
|
Under the influence of? 2
|
TGF-b, IL-6
|
|
|
Function? 2
|
Recruit neutrophils, macrophages
|
|
|
Treg markers? 5
|
CD29, CD28, CD25, CD4, foxP-3
|
|
|
Induced by? 1
|
TGF-b
|
|
|
Secrete?
|
TGF-b, IL-10
|
|
|
Function? 2
|
Suppress T cell response; Ag-specific
|
|
|
4 characteristics that make a good immunogen
|
Foreign; antigenic; susceptible to degradation; >40 kD
|
|
|
Acute phase responses: 9
|
Fever, appetite loss, slow sleep wave, Neutrophil release, steroid release, hemo fx of shock, CRP, DIC, incr fibrinogen
|
|
|
Define haptogen
|
Soluble particle that does not allow cross-linking
|
|
|
Titer definition?
|
1/highest dilution that gives + agglutination
|
|
|
Define particulate
|
Insoluble particle that allows agglutination but not precipitation
|
|
|
Gene responsible for rearrangements in TCR, BCR?
|
RAG genes
|
|
|
Gene responsible for neg selection?
|
AIRE gene
|
|
|
Lost in which disease?
|
APS-1
|
|
|
Direct v. indirect Coombs test?
|
Direct uses baby's RBC; Indirect uses mother's Ab
|
|
|
Location of T v. B cell in:
|
|
|
|
LN
|
T: paracortex, B: germinal center, cortex
|
|
|
Spleen
|
T: PALS, B: lymph nodule
|
|
|
Tonsil
|
T: periphery, B: germinal center
|
|
|
Cells that carry MHC I? 2
|
All except sperm, RBC
|
|
|
Cells that carry MHC II?
|
APC
|
|
|
Pathway for making MHC I?
|
Endogenous
|
|
|
Pathway for making MHC II?
|
Exogenous
|
|
|
HLA inv in MHC I? 3
|
A, B, C
|
|
|
HLA inv in MHC II? 3
|
DP, DQ, DR
|
|
|
Which MHC requires b2?
|
MHC I
|
|
|
Purpose of junctional flexibility?
|
Ab diversity; imprecise joinging of segments
|
|
|
When does somatic hypermutation occur?
|
After Ag stimulate in the germinal center
|
|
|
Disadvantages of T-independent response? 5
|
Not robust; no memory; only IgM; lower affinity; lag time
|
|
|
What Ag stimulates T-independent response? 2
|
Polysaccharides with repeating epitope, LPS
|
|
|
Cytokine signal transduction via? 1
|
JAK/STAT:
|
|
|
Process? 5
|
R dimerization -> JAK kinases -> phophorylated tyr on STAT -> dimerization -> translocate to nucleus
|
|
|
Suppression of cytokine signaling by?
|
SOCS proteins (the initials)
|
|
|
Autoimmune Dz: Target AutoAg, effector cell
|
194
|
|
|
Graves dz 1, 1
|
TSH-R; B cell/autoAb
|
|
|
Myasthenia gravis 1, 1
|
ACh-R; B cell/autoAb
|
|
|
Pernicious anemia 2, 1
|
Gastric parietal cell, IF; B cell/autoAb
|
|
|
ANCA-assoc vasculitides 1, 1
|
MPO serine proteinase; B cell/autoAb
|
|
|
Autoimmune hemolytic anemia 1, 1
|
Rh blood group Ag; B cell/autoAb
|
|
|
Idiopathic thromobocytpenic purpura (ITP) 2, 1
|
Platelet membrane proteins, integrin; B cell/autoAb
|
|
|
SLE 2, 1
|
ds DNA histone, snRNPs; B cell/autoAb
|
|
|
Sjogren Syndrome 4, 1
|
Salivary duct Ag, SS-A, SS-B, nucleoproteins; B cell/autoAb
|
|
|
Scleroderma 4, 1
|
Centromeric proteins in fibroblast, nucleolar Ag, IgG, Scl-70; B cell/autoAb
Unknown effector cells |
|
|
Pemphigus vulgaris 1, 1
|
Desmoglin 3; B cell/autoAb
|
|
|
Goodpasture Syndrome 2, 1
|
Renal & lung basement membrane collagen type IV; B cell/autoAb
|
|
|
Rheumatoid arthritis 2, 3
|
Unknown cartilage Ag, IgG; TH, CTL, B cell/autoAb
|
|
|
Hashimoto's thyroiditis 3, 2
|
Thyroid proteins: thyroglobulin, microsomal, TPO
TH, B cell/autoAb |
|
|
T1DM 1, 2
|
Pacreatic β cell Ag; TH, B cell/autoAb
|
|
|
MS 1, 1
|
Myelin basic protein; TH
|
|
|
Central tolerances occurs where? 2
|
Bone marrow for B cells; Thymus for T cells
|
|
|
Peripheral tolerance occurs where?
|
Peripheral lymphoid organs
|
|
|
Anergy mechanism?
|
When an Ag matches BCR/TCR but lacks costimulatory interactions
|
|
|
Major mechanism for peripheral tolerance?
|
Anergy
|
|
|
Difference between low dose and high dose tolerance to food Ag? 2
|
Low-dose induce T cell suppression; High-dose induce T cell anergy or deletion
|
|
|
Lymph node
|
200
|
|
|
Follicles contain which cell?
|
B
|
|
|
Paracortex contain which cell?
|
T
|
|
|
Medullary sinus contain which cell?
|
Macrophage
|
|
|
Medullary cord contain which cell?
|
Plasma
|
|
|
What part enlarges in lymphadenopathy?
|
Paracortex
|
|
|
Where do upper limbs, lateral breast drain?
|
Axilla
|
|
|
Where does stomach drain?
|
Celiac
|
|
|
Duodenum, jejunum?
|
Sup mesenteric
|
|
|
Sigmoid colon?
|
Colic -> inf mesenteric
|
|
|
Rectum?
|
Iliac
|
|
|
Anal cana, scrotum, thigh?
|
Superficial inguinal
|
|
|
Testes?
|
Para-aortic
|
|
|
Lateral side of dorsum of foot?
|
Popliteal
|
|
|
Right lymphatic duct drains? 2
|
R arm, R half of head
|
|
|
Spleen
|
201
|
|
|
Red pulp contain?
|
RBC
|
|
|
T cells located in? 2
|
PALS, White pulp
|
|
|
Margizonal zone contain?
|
Macrophage
|
|
|
Follicles contain which cell?
|
B cell
|
|
|
Purpose of macrophages in spleen? 2
|
Eat RBC, encapsulated bact
|
|
|
Signs of postsplenectomy? 4
|
Howell-Jolly bodies, Target cells, thrombocytosis, susceptible to encapsulated bact
|
|
|
Thymus
|
201
|
|
|
Derived from which pouch?
|
3rd
|
|
|
positive selection occurs at?
|
cortico-medullary jxn
|
|
|
Negative selection occurs at?
|
cortico-medullary jxn
|
|
|
Cortex contains?
|
Immature T cells
|
|
|
Medullay contain?
|
Mature T cell
|
|
|
HLA subtypes assoc with dz
|
202
|
|
|
A3 1
|
hemochromatosis
|
|
|
B27 4
|
Psoriasis, Anklyosing spondylitis, IBD, Reiter's (PAIR)
|
|
|
B8 1
|
Grave's
|
|
|
DR2 4
|
MS, hay fever, SLE, Goodpasture's
|
|
|
DR3 1
|
T1DM
|
|
|
DR4 2
|
RA, T1DM
|
|
|
DR5 2
|
Pernicious anemia, Hashimoto's
|
|
|
DR 7 1
|
Steroid-responsive nephrotic syndrome
|
|
|
Type 1 HSR dz? 4
|
Anaphylaxis: bee sting, food allergy, drug allergy
Allergic/atopic: rhinitis, hay fever, hives Eczema Asthma |
|
|
Type 2 HSR dz? 11
|
Acute hemolytic transfusion rxn
Bullous pemphigoid Erythroblastosis fetalis Goodpasture's syndrome Grave's dz Hemolytic anemia ITP Myasthenia gravis Pemphigus vulgaris Pernicious anemia Rheumatic fever |
|
|
Type 3 HSR dz? 7
|
Arthrus rxn
Hypersensitivity pneumonitis Polyarteritis nodusum Post-strep glomerulonephritis Rheumatoid arthritis Serum sickness SLE |
|
|
Type 4 HSR dz? 7
|
Constact dermatitis
Guillain-Barre GVHD Hashimoto's thyroiditis MS PPD T1DM |
|
|
AutoAb & assoc dz
|
212
|
|
|
ANA
|
SLE, nonspecific
|
|
|
anti-dsDNA, anti-Sm
|
SLE
|
|
|
Anti-histone
|
Drug-induced lupus
|
|
|
Anti-IgG (RF)
|
RA
|
|
|
Anticentromere
|
CREST
|
|
|
anti-Scl70 (anti-DNA topo 1)
|
diffuse scleroderma
|
|
|
anti-mitochondrial
|
primary biliary cirrhosisi
|
|
|
anti-gliadin, anti-endomysial
|
Celiac dz
|
|
|
anti-basement membrane
|
Goodpasture
|
|
|
Anti-desmoglein
|
Pemphigus vulgaris
|
|
|
antimicrosomal, antithyroglobulin
|
Hashimoto's
|
|
|
anti-Jo-1
|
polymyositis, dermatomyositis
|
|
|
anti-SS-A (anti-Ro)
|
Sjorgren's
|
|
|
Anti-SS-B (anti-La)
|
Sjorgren's
|
|
|
Anti-U1 RNP (ribonucleoprotein)
|
Mixed CT dz
|
|
|
Anti-smooth muscle
|
Autoimmune hepatitis
|
|
|
anti-glutamated decarboxylase
|
T1DM
|
|
|
c-ANCA
|
Wegner's granulomatosis
|
|
|
p-ANCA
|
Vasculitides
|
|
|
Bruton's agammaglobulinemia
|
213
|
|
|
Genetics?
|
X-linked R
|
|
|
Defect in?
|
BTK; blocks B cell differentiation
|
|
|
Presentation?
|
Recurrent bact inf after 6m from low opsonization
|
|
|
Labs 4
|
Norm pro-B; low matureation; low # B cells; low Ig
|
|
|
Hyper-IgM syndrome
|
213
|
|
|
Defect in?
|
CD40L; can't class switch
|
|
|
Presentation?
|
Severe pyogenic inf early in life
|
|
|
Labs 4
|
High IgM, low IgG, low IgA, low IgE
|
|
|
Selective Ig def
|
213
|
|
|
Defect in? 2
|
Isotype switching; specific class low
|
|
|
Presentation?
|
Sinus & lung inf, milk allergies, diarrhea, anaphylaxis to IgA expo
|
|
|
Labs 1
|
IgA def common
|
|
|
CVID = common variable immunodef
|
213
|
|
|
Defect in?
|
B cell maturation
|
|
|
Presentation? 4
|
Can be in 20s-30s; incr risk autoimmune dz, lymphoma, sinopulm inf
|
|
|
Labs 3
|
Norm # B, low plasma cells, low Ig
|
|
|
Thymic aplasia (DiGeorge)
|
213
|
|
|
Genetics?
|
22q11 deletion
|
|
|
Defect?
|
Failure to develop 3rd & 4th pharyngeal pouches
|
|
|
Presentation?
|
Tetany, recurrant viral/fungal inf, congenital heart and great vessel defects
|
|
|
Labs 4
|
Low # T cells, low PTH, low Ca, absent thymic shadow
|
|
|
IL-12-R def
|
213
|
|
|
Defect?
|
Low TH1 response
|
|
|
Presentation?
|
Disseminated mycobact inf
|
|
|
Lab 1
|
low IFN-g
|
|
|
Hyper-IgE syndrome = Job's syndrome
|
213
|
|
|
Defect
|
Low IFN-g -> neutrophils not respond to chemotactic stim
|
|
|
Presentation: FATED
|
course Facies, cold staphylococcal Abscesses, retained primary Teeth, incr IgE, Derm problems (eczema)
|
|
|
Lab
|
High IgE
|
|
|
Chronic mucocutaneous candidias
|
213
|
|
|
Defect?
|
T cell dysfxn
|
|
|
Presentation?
|
Mucocutaneous candida inf
|
|
|
SCID
|
214
|
|
|
Defects? 3
|
IL-2R, adenosine deaminase def, failure to make MHC II Ag
|
|
|
Presentation?
|
Recurrent inf
|
|
|
Tx?
|
Bone marrow transplant
|
|
|
Labs? 4
|
Low IL-2R; low # T cell; incr adenine; low dNTPs
|
|
|
Ataxia-telangiectasia
|
214
|
|
|
Defect?
|
DNA repair enzymes
|
|
|
Presentation: triad
|
cerebellar defect, spider angiomas, IgA def
|
|
|
Lab? 1
|
IgA def
|
|
|
Wiskott-Aldrich syndrome
|
214
|
|
|
Genetics?
|
X-linked R
|
|
|
Defect?
|
Progressive deletion B & T cells
|
|
|
Presentation: triad, TIE
|
Thromobocytopenia purpura, Inf, eczema
|
|
|
Labs 3
|
High IgE, high IgA, low IgM
|
|
|
LAD1
|
214
|
|
|
Defect?
|
LFA-1 (CD18) on phagocytes
|
|
|
Presentation? 3
|
Recurrent bact inf, absent pus formation, delayed separation of umbilicus
|
|
|
Lab
|
Neutrophilia
|
|
|
Chediak-Higashi syndrome
|
214
|
|
|
Genetics?
|
Auto R
|
|
|
Defect?
|
Microtubular fxn
|
|
|
Presentation 3
|
Recurrent pyogenic inf by staph & strep; partial albinism; peripheral neuropathy
|
|
|
Chronic granulomatous dz
|
214
|
|
|
Defect?
|
Lack NADPH
|
|
|
Presentation?
|
Incr susceptibility to catalase + org
|
|
|
Lab? 1
|
Neg nitroblue tetrazolium dye reduction test
|
