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Immunology Flashcards

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1
Q

X-linked (Bruton) agammaglobulinemia

A

Defect in BTK, a tyrosine kinase gene which results in no B cell maturation. XR. Common in Boys. Recurrent bacterial and enteroviral infections after 6 months old. **dec Ig of all classes, absent/scant lymph nodes. **Normal CD19+ B cell count, dec pro-B.

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2
Q

Selective IgA deficiency

A

Most common 1° immunodeficiency. Asymptomatic, can see Airway and GI infections, Autoimmune disease, Atopy, Anaphylaxis to IgA-containing products. IgA<7mg/dL with normal IgG and IgM levels

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3
Q

Common variable immunodeficiency

A

defect in B-cell differentiation. Acquired in 20-30s, inc risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infection. Dec plasma cells and immunoglobulins

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4
Q

Thymic aplasia (DiGeorge Syndrome)

A

22q11 deletion; failure to develop 3rd and 4th pharyngeal pouch, absent thymus and parathyroids. Tetany (hypocalcemia), recurrent viral/fungal infections (T cell deficiency), conotruncal abnormalities (tetrology of fallot, truncus arteriosus). Dec T cells, dec PTH, dec Ca2+. **Absent thymic shadow on CXR, FISH detection of 22q11 deletion. **

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5
Q

IL-12 receptor deficiency

A

dec TH1 response. AR. Disseminated mycobacterial and fungal infections; may present after administration of BCG vaccine. decreased IFN-y (secreted by TH1 cells)

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6
Q

Autosomal dominant hyper-IgE syndrome (Job syndrome)

A

deficiency of TH17 cells due to STAT3 mutations so impaired recruitment of neutrophils to sites of infection. FATED: coarse Facies, cold (noninflamed) staphylococcal Abscesses, retained primary Teeth, inc IgE, Dermatologic problems (eczema). inc IgE and dec IFN-y

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7
Q

What is the role of IFN-y?

A

Secreted from TH1 cells and activates macrophages. Important in granuloma formation and maintainene.

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8
Q

Severe combined immunodeficiency (SCID)

A

defective IL-2R gamma chain or adenosine **deaminase deficiency. **Filaure to thrive, chronic diarrhea, thrush. Recurrent viral, bacterial, fungal, and protozoal infections. Dec T cell receptor excision circles (TRECs). Absense of thymic shadow, germinal centers and T cells on flow cyto,etru. Tx: bone marrow transplant (no concern for rejection, yippee)

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9
Q

Wiskott-Adlrich syndrome

A

mutation in WAS gene, T cells unable to reorganize actin cytoskeleton. WATER: Wiskott-Aldrich, Thrombocytopenic purpura, Eczema, Recurrent infection. Increased risk of autimmune diseas and malignancy. inc IgE and IgA, low/normal IgG and IgM

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10
Q

Chronic Granulomatous Disease

A

defective NADPH oxidase, dec ROS. Suseptible to PLACESS: Pseudomonas, Listeria, Aspergillus, Candida, E.coli, Serratia, Staph aureus (nocardia, burkholderia cepacia)

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11
Q

What drains to the para-aortic lymph nodes?

A

testes, ovaries, kidneys and uterus

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12
Q

What drains to the internal iliac lymph nodes?

A

lower rectum to anal canal (above pectinate line), bladder, vagina and prostate

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13
Q

What drains to the popliteal lymph nodes?

A

dorsolateral foot, posterior calf

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14
Q

What drains to the axillary nodes?

A

upper limb, breast, and skin above the umbilicus

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15
Q

What enters the right lymphatic duct?

A

drains right side of body above the diaphragm

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16
Q

What does the thoracic duct drain?

A

everything that the right lymphatic duct doesn’t get, and enters in the left subclavian and internal jugular veins.

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17
Q

Why are asplenic patients at higher risk for encapsulated bacterial infections?

A

No spleen means less IgM production and decreased complement activation therefore less C3b opsonization of encapsulated bacteria

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18
Q

What are the encapsulated bacteria that asplenic patients are suspetible to?

A

SHiNE SKiS (or Even Some Pretty Nasty Killers Have Shiny Bodies):

Streptococcus pneumoniae

H. influ

N. meningitidis

E. coli

Salmonella

Klebsiella pneumoniae

Group B Strep

Klebsiella

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19
Q

Which bacteria from that infect asplenic patients have vaccines?

A

S. pneumo, H. flu type B, and N. meningitidis

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20
Q

What pharyngeal pouch is the thymus from?

A

3rd pharyngeal pouch!

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21
Q

HLA-A3 association?

A

Hemochromatosis

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22
Q

HLA-B27 assocations?

A

PAIR.

Psoriatic arthritis

Ankylosing spondulitis

IBD arthritis

Reactive arthritis

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23
Q

HLA-DQ2/DQ8 associations?

A

celiac disease

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24
Q

HLA-DR2 assocations?

A

MS, hay fever, SLE, Goodpasture syndrome

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25
HLA-DR3 associations?
DM1, SLE, Graves disease
26
HLA-DR4 assocations?
Rheumatoid arthritis, DM1 (**4** walls in a **rheum**)
27
HLA-DR5 associations?
pernicious anemia leads to B12 deficiency, hasimoto thyroiditis
28
What is the role of TNF-alpha in granuloma formation?
Secreted from macrophages and maintains the granuloma. If on anti-TNF therapy then need to first make sure they don't have TB, or else it will become reactivated and disseminated.
29
What receptor recognizes LPS of gram - bacteria?
CD14 (TLR4) on macrophages
30
What results from TLR activation via recognition of a PAMP?
upregulation os NF-KB whihch is a nuclear transcription factor that activates immune response genes leading to production of multiple immune mediators
31
What 4 key mediators activate neutrophils?
1. LTB4 2. C5a 3. IL-8 4. bacterial products
32
Action of PGI2, LTD4, and PGE2?
mediate vasodilation @ arteriole and increased vascular permeaility @ post-capillary venule
33
Action of LTC4, LTD4, and LTE4?
vasoconstriction, **bronchospasm** and increased vascular permeability
34
How are mast cells activate?
1. tissue trauma, 2. complement C3a and C5a, and 3. cross-linking of cell-surface IgE by antigen
35
Main action of luekotrienes?
part of the delayed response to inflammtion and mainstains the acute infalmmatory response
36
How is the classicla pathway activated?
C1 binds IgG or IgM (**GM** makes **classic** cars)
37
What components make up MAC?
C5b-C9
38
Describe the kinin system?
Activated by Factor XII, and then Kinin cleaves high-molecular weight kinogen to **bradykinin** which is a **vasodilator** and increases vascular permiability and causes **pain**.
39
What enzymes lead to production of prostaglandins?
cycloxygenase
40
What enzyme leads to production of leukotrienes?
5-lipoxygenase
41
etiology of a fever?
pyrogens (LPS) cause macrophages to release IL-1 and TNF which increases COX activity so increased PGE2 production which raises the temp set point.
42
Factors involved in margination?
vasodilation resulting in slower blood flow allowing neutrophils to flow on the periphery
43
Factors involved in Rolling of neutrophils?
**P-selectin** released from **Weibel-Palade bodies** in endothelial cells and **E-selectin** induced by TNF and IL1. These selectins bind **sialyly Lewis X** on neutrophils
44
Factors invovled in firm adhesion of neutrophils?
**ICAM** and **VCAM** on endothelial cells by TNF and IL1. **Intigrins** are upregulared on leukocytes by C5a and LTB4
45
Factors invovled in transmigration and chemotaxis?
migrate across postcapillary venules and more toward injury via **IL-8, C5a, LTB4 and bacterial products**
46
How do you differnetiate between CGD caused by NADPH oxidase deficiency or MPO deficiency?
NADPH oxidase = negative Nitroblue tetraolium test MPO= positive NBT test (turns blue)
47
MPO deficiency puts you at an increased risk for what infections?
Candida
48
What is the time frame in acute inflammation for Neutrophils and macrophages?
Neutrophils appear in the first 24 hours then disapear. Macrohpages predominatne 2-3 days after initiation of inflammation
49
If you see pus, what type of inflammation is occuring?
acute inflammation
50
Do macrophages predominantly use O2 dependent or independent killing?
O2-independent killing (lysozyme)
51
What are the main cell types of acute inflammation vs. chronic inflammation?
acute = neutrophils and macrophages (innate immunity) chronic = lymphocytes and plasma cells (adaptive immunity)
52
How are CD4 helper cells activated?
MHCII on APCs presents the antigen to CD4 cells and then **B7** on APC interacts with **CD28** on CD4 helper cells.
53
What do TH1 cells secrete?
**IFN-y** (activated macrophages, promotes B cell class-switching to IgE), **IL-2**
54
What do TH2 cells secrete?
**IL-4** (B-cells make switch to make IgE), **IL-5, IL-6, IL-13** (similar function to IL-4)
55
What provides the second signal in TH1 cell activation?
IL-2
56
What is the role of caspase?
pathway involved in apoptosis that is activated by CD8 T cells.
57
CD40 and CD40L
CD40 receptor is on B cells and bind CD40L, involved in activation of B cells and inducing class-switching
58
What are epitheliod histiocytes?
macrophages with abundant pink cytoplasm that are the defining cells of a granuloma
59
Noncaseating Granulomatous inflammation characteristics?
**NO CENTRAL NECROSIS**, usually due to sarcoidosis, beryllium expsures, Crohn disease, and cat scatch disease
60
Caseating Granulomatous inflammatory characteristics?
**central necrosis** and is characteristic of tuberculosis and fungal infections.
61
Steps in granuloma formation? (for both caseating and non-caseating)
1. macrophages present antigen to TH1 cells 2. macrophages secrete IL-12 which induces CD4 helper T cells to differentiate into TH1 cells. 3. TH1 cells secrete IFN-y which converts macrophages to epitheliod histiocytes and giant cells
62
What are patients with SCID suseptible to?
fungal, viral, bacterial, and protozola infections
63
What typeos of vaccines must be avoided in Brutons Agammaglobulinemia?
live vaccines (polio)
64
C1 esterase inhibitor deficiency?
results in hereditary angioedema, which is characterized by edema of the skin, **especially periorbital. **Increases bradykinin (ACEi contraindicated)
65
What are the permanent tissues?
myocardium, skeletal muscle, and neurons. Lack significant regenerative potential
66
What are the labile tissues?
tissues that posess stem cells that **continuously** cycle to regnerate capacity of tissue. stem cells in mucosal crypts, basal layer of skin, and hematopoietic stem cells in bone marrow
67
What are the stable tissues?
Tissues made of cells that are quiescent (G0) but can reenter the cell cycle when necessary. **Liver **and **kidney**
68
What is collagenase?
removes type III collagen from granulation tissue and uses **zinc** as a cofactor.
69
What type of collagen is a hypertrophic scar made of?
type I collagen
70
What type of collagen is a keloid made of?
type iII collagen
71
where does hydroxylation of proline and lysine procollagen occur and what is the cofactor?
Occurs in the RER and **vitamin C** is the cofactor
72
Where does cross-linking of collagen occur and what is the necessary cofactor?
occurs extracellularly and **copper** is the cofactor for **lysyl oxidase** which performs the cross-linking between lysine and hydrozylysine. Deficiey in this process = **ehlers-danlos**
73
C3 deficiency?
increased risk of severe, recurrent pyogenic sinus an (S. pneumo and H. flu), increased susceptibility to type II hypersensitivity reactions
74
What are the acute phase cytokines?
IL-1, IL-6 and TNF-alpha
75
Function of TNF-alpha?
mediates septic shock, activates endothelium, leukocyte recruitment, maintains granulomas.
76
What are the common passive antibodies that are given to patients?
**T**o **B**e **H**ealed **R**apidly **T**etanus toxin **B**otulinum toxin **H**BV **R**abies

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