Term
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Definition
CD14 is a TLR that recognizes LPS (a PAMP)
TLRs upregulate NF-kB --> production of immune mediators
TLRs are present on both acute & chronic inflammatory cells |
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Term
| what PGL mediates fever & pain |
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Definition
PGE2
Fever: IL1 & TNF increase COX activity in anterior hypothalamus --> PGE2 resets setpoint |
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Term
| which prostaglandins mediate arteriolar dilation & increased postcapillary venule permeability |
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Definition
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Term
| Name important chemoattractants for neutrophils (5) |
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Definition
1) C5a
2) IL8 (secreated by Mphages)
3) LTB4
4) bacterial products (e.g. N-formyl-methionine) |
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Term
| slow reacting substance of anaphylaxis |
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Definition
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Term
| Leukotrienes that mediate vasoconstriction, bronchospasm, and increased vascular permiability (remember, always in the postcapillary venule) |
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Definition
LTC4, LTD4, LTE4
(LTB4 is an important neutrophil chemotactic factor) |
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Term
| 3 things that activate mast cells |
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Definition
1) C3a & C5a (anaphylatoxins)
2) Trauma
3) x-linking IgE |
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Term
| Acute & delayed response to histamine |
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Definition
Acute: vasodilation & increased permeability of vasculature
Delayed: increased production of AA products (esp leukotrienes) |
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Term
| what 2 antibodies activate complement |
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Definition
IgG & IgM; bind and activate C1 through classical pathway (GM makes classic cars)
C1 --> C1C4b --> C4bC2b --> C4bC2bC3b
(mannose-binding lectin pathway is exactly the same but has MBL instead of C1/Ig) |
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Term
Name role of each:
1) C3a & C5a
2) C5a
3) C3b |
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Definition
1) anaphylatoxins --> trigger mast cell degranulation
2) neutrophil chemotaxis (& upregulation of leukocyte integrins, along w/ LTB4)
3) opsonization |
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Term
| 2 mediators of pain & how do they do it? |
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Definition
| PGE2 & Bradykinin --> sensitize sensory nerve endings |
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Term
Factor XII is an important activator of both the inflammatory (complement) and coagulation systems.
In sepsis, esp with G(-) organisms, XII is activated by LPS & mediates what very serious event? |
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Definition
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Term
| Weibel-Palade bodies have what 2 important things in them (remember, one is W and one is P) |
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Definition
VonWillibrand factor & P-selectin (rolling)
WP bodies are seen inside endothelial cells |
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Term
Name proteins involved in:
1) Rolling
2) Adhesion |
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Definition
1) E-selectin (induced via IL1 & TNF) & P-selectin (WP bodies) on endothelium; Sialyl-Lewis X on leukocytes
2) ICAM1 (induced via TNF, IL1) on endothelia; LFA1 (induced via C5a, LTB4) on leukocytes |
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Term
| What is the most common cause of leukocyte adhesion deficiency? |
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Definition
| CD18 (Beta subunit of many integrins including LFA1) |
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Term
- pyogenic infections
- neutropenia
- giant granules in leukocytes
- defective hemostasis (abnormal platelet function)
- Albinism
- Peripheral neuropathy |
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Definition
| Chediak Higashi syndrome (LYST gene, defect in lysosomal trafficking; de-facto defect in microtubules) |
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Term
- pyogenic infections
- neutropenia
- giant granules in leukocytes
- defective hemostasis (abnormal platelet function)
- Albinism
- Peripheral neuropathy |
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Definition
| Chediak Higashi syndrome (LYST gene, defect in lysosomal trafficking; de-facto defect in microtubules) |
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Term
| Chronic granulomatous disease. What is deficient and inheritance pattern? |
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Definition
NADPH oxidase (can't make superoxide)
X-linked |
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Term
| Important catalase positive bugs in CGD |
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Definition
S.aureus
Pseudomonas cepacia (pulmonary)
Serratia marensence
Aspergillus
Nocardia (branched, acid fast, pulmonary, use sulfa) |
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Term
O2 --> O2* --> H2O2 --> HOCl
Which step does the nitroblue tetrazolium test identify?
If you are MPO deficient, what bug are you more susceptible to? |
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Definition
- NBT tests for NADPH oxidase (i.e. O2 --> O2*); it is negative in CGD but positive in MPO deficiency
- MPO deficiency may lead to increased candida infections (esp in diabetics) |
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Term
Macrophages secrete IL10 & TGF-Beta to cause what?
They secrete IL8 for what? |
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Definition
resolution and healing of site of acute inflammation
IL8 secreted to further recruit neutrophils & continue acute reaction |
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Term
| What cell is responsible for the formation of an abscess? |
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Definition
| macrophages: they wall off cavity |
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Term
| Activation of T-cells requires binding MHC (I or II for CD8 or CD4) and a second signal. Name the second signal for each CD4+ & CD8+ |
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Definition
- CD4+: B7 on APCs bind CD28 on CD4+ --> activate cell to Th1 or Th2:
Th1: secrete IL2 & IFN-gamma (T-cell growth factor/CD8+ activator & Mphage activator)
Th2: secretes IL4 (IgG/IgE class switch), IL5 (eosinophil prod/chemotaxis, IgA class switch), IL10 (inhibit Th1)
- CD8: IL2 from CD4+ Th1 provide 2nd signal --> secrete perforin & granzyme OR FASL expression --> activation of caspases --> apoptosis of target cell
ASIDE: CD28 binds B7 & activates CD4; CD152 (CTLA4) binds B7 and inactivates T-cells |
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Term
What do B-cells need to secrete IgM?
What do B-cells need to class switch? |
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Definition
IgM: B-cells just need their surface Ig (IgM or IgD) to bind antigen
Class switch: must bind antigen & receive costimulation from CD4+ Th cell via interaction with MHCII-TCR & CD40-CD40L) --> IL4,5 secreted by CD4 |
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Term
| Defining features of granulomas |
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Definition
epithelioid histocytes (macrophages w/ dense, eosinophilic cytoplasm)
Histiocytic conversion is induced by IFN-gamma secretion by Th1 cells (which is induced by IL12 secretion by macrophages, which is induced by MHCII-TCR interaction w/ CD4 cells) |
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Term
Name if granuloma is caseating or non-caseating:
1) Cat scratch disease
2) sarcoidosis
3) TB
4) Beryllium
5) Crohns
6) Fungal infection
7) Foreign materia (e.g. silicon from breast implant) |
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Definition
1) NC ("stellate-shaped" with central abscess)
2) NC (asteroid body)
3) C
4) NC
5) NC
6) C
7) NC
"caseation" is a characteristic of TB & Fungal granulomas (everything else will generally be non-caseating) |
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Term
| Which pharyngeal pouches don't develop in DiGeorge? |
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Definition
3rd & 4th
Remember "CATCH-22": cardiac, abnormal facies, thymic aplasia, cleft palate, hypocalcemia, 22q11 deletion
Multiple abnormalities of heart/great vessels may exist |
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Term
Name structures derived from each pharyngeal pouch:
1st:
2nd:
3rd (dorsal wing):
3rd (ventral wing):
4th: |
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Definition
1st: inner ear, eustacian tube, etmoid air cells
2nd: epithelial covering of palantine tonsils
3rd (dorsal wing): Inf parathyroids
3rd (ventral wing): Thymus
4th: Sup parathyroids |
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Term
3 causes of SCID (& inheretance)
(SCID is a combined humoral & cell-mediated defect) |
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Definition
1) IL receptor defect (esp IL2): X-linked
2) Adenosine deaminase deficiency: AR, buildup of dATP --> inhibits ribonucleotide reductase & is toxic --> decreased lymphocyte prolif
3) MHCII deficiency: AR; CD4+ can't activate CD8+ or B-cells
NOTE: DON'T give these kids live-attenuated vaccines (e.g. MMR, VZV, Sabin, Yellow fever, Rabies, Seasonal flu nose spray) |
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Term
| X-linked agammaglobulinemia etiology & susceptibility |
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Definition
bruton's tyrosine kinase (X-linked); usually Px ~6mo after maternal IgA is gone
susceptible to recurrent bacterial, enterovirus (e.g. polio/coxsackie), & Giardia infections (remember these)
DON'T give live-attenuated vaccines (e.g. Sabin, MMR, etc) |
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Term
| Same Px as Brutons agammaglobulinemia, except it presents in late childhood/adulthood & it's not X-linked |
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Definition
Common Variable Immunodeficiency; caused by EITHER a T-cell OR B-cell dysfunction leading to low Ig
Same as BA: recurrent infections w/ bacterial, enterovirus, & giardia |
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Term
| most common immunodeficiency? |
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Definition
| Selective Ig deficiency (usually IgA); anaphylaxis w/ transfusions (exposure to IgA); Celiac association |
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Term
|
Definition
Hyper IgM:
- all other Ig are low
- Severe pyogenic infections (esp mucosal) early in life (can't opsonize)
- normal T & B cell levels |
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Term
|
Definition
Thrombocytopenia, Eczema, Infections (TIE)
caused by WASP; X-linked (increased IgE & IgA; decreased IgM) |
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Term
| Immunoglobulin deficient in Ataxia-Telangiectasia |
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Definition
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Term
| Endocarditis involving both sides of the mitral valve |
|
Definition
| Libman-Sacks endocarditis: seen in SLE; Immune complex deposits & creates local inflammation on valve |
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Term
| 2 common causes of death in SLE |
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Definition
1) Renal failure (most commonly the nephritic "diffuse proliferative glomerulonephritis")
2) infections |
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Term
|
Definition
1) ANA (most sensitive)
2) anti-dsDNA
3) anti-Smith (most specific) |
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Term
| 4 drugs causing drug-induced SLE |
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Definition
| procainamide, hydralazine, INH, phenytoin |
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Term
Type of hypersensitivity in Sjogrens?
What is the autoantibody? |
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Definition
Type IV (lymphocyte mediated damage w/ fibrosis)
Anti-SS-A & Anti-SS-B (Ro & La): RNPs (splicesome) |
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Term
| Rapid, unilateral expansion of a parotid glands of a pt w/ Sjogrens |
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Definition
| B-cell (marginal zone) lymphoma |
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Term
| Autoantibody in Scleroderma? CREST? |
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Definition
- Anti-Scl70 (topoisomerase I)
- Anti-Centromere |
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Term
| Autoantibody in mixed connective-tissue disease? |
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Definition
Anti-U1 ribonucleoprotein
(shows mixed features of SLE, SS, polymyositis; may eventually progress to full-blown SLE or SS) |
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Term
| Where are the stem cells located in the GI system |
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Definition
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Term
| CD marker for hematopoietic stem cells |
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Definition
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Term
Define:
1) Labile tissue
2) Stable tissue
3) Permanent tissue |
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Definition
1) Continuously cycle & regenerate tissue (e.g. skin basal cells, intestinal crypt cells)
2) Stay in G0 unless they need to regenerate tissue (e.g. hepatocytes, proximal tubule cells in ATN)
3) Can't regenerate --> always replaced w/ scar (e.g. myocardium, Skeletal muscle, Neurons) |
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Term
| Type of collagen in granulation tissue? What is it replaced by? |
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Definition
Type 3 (very pliable)
Type 1 (strong) |
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Term
| What type of collagen is seen in keloids |
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Definition
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Term
Name cofactor needed for each enzyme:
1) Collagenase (dissolves T3 collagen in preparation to be replaced by T1)
2) Proline/lysine hydroxylase
3) Lysyl oxidase (x-link collagen) |
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Definition
1) Zn
2) Vitamin C
3) Cu
if any of these cofactors are missing, there will be delayed wound healing |
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Term
| Most common bacteria causing delayed wound healing? |
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Definition
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