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Things I forget most Flashcards

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1
Q

What causes Autoimmune Polyendocrine syndrome?

A

AIRE deficiency.. essentially its an incomplete tolerance in the thymus

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2
Q

what causes IPEX syndrome?

A

FoxP3 deficiency i.e. impaired regulatory t cells

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3
Q

What 3 molecules associated with peripheral B cell tolerance are responsible for inhibition… and if they have defects.. will result in autoimmunity?

A

CD22 inhibitory receptor, Lyn, SHP1

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4
Q

which region is changed during BCR editing?

A

light chain of the Ig

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5
Q

How are T cells involved in metabolic destruction?

A

CD25

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6
Q

How do Treg cells inhibit DCs?

A

blocking CD80/86 on DCs using their CTLA4

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7
Q

What’s the full name of Treg cells?

A

CD4+CD25+CTLA4+ Treg cells

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8
Q

What does AIRE stand for and what happens when it is deficient?

A

AutoImmune Regulator

Helps for negative selection of T cells in the thymus. It’s a transcription factor.

When it’s not there, there are decreased expression of self-ags in the thymus, which means self reactive T cells are not eliminated. causes injury because they move into other places and cause injury

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9
Q

What does CTLA4 do extrinsically? Intrinsically?

A

Intrinsic = directly acts on the cell to tell it to stop

Extrinsic = Tells helper T cells or Treg cells to do stuff to B cells

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10
Q

What causes chronic autoimmunity?

autoimmunity in general?

A

T cells and B cells that are active and doing shit without ongoing infection or other discernable causes

failure of self-tolerance

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11
Q

What is the steps of autoimmunity?

How do you have a clinical manifestation?

A

Genetic susceptibility

Failure of self tolerance

Functional self-reactive lymphocytes

THEN

activation of self reactive lymphocytes and immune response against self tissues

clinical manifestation NEEDS an environmental trigger. (infections, tissue injury)

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12
Q

What are the 3 environmental triggers?

A

Molecular Mimicry
Polyclonal activation
release of previously sequestered Ags

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13
Q

What environmental trigger is associated with Rheumatic Fever, and MS?

A

Molecular Mimicry

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14
Q

What noninfectious trigger can induce MS and SLE in certain people?

A

blockade of TNF-alpha

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15
Q

Which avidity B cell is given a second chance?

A

High avidity

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16
Q

Type 1: What is the main mediator and what creates this?

What’s its function?

A

igE

Mast Cells

Immediate response

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17
Q

Type 2: What is the main mediator and what does it respond to?

A

IgG and IgM and it responds to Ags in the TISSUES and cause complement-dependent tissue injury and disease

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18
Q

Type 3: What is the main mediator and what does it respond to?

A

IgG and IgM and it responds to CIRCULATING ags to form immune complexes, which deposit in vessels and cause complement-dependent injury in the vessel wall

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19
Q

Type 4: What is the main mediator and what does it respond to?

A

T cells and results from inflammation caused by CYTOKINES produced by CD4TH1 and CD4 TH17 cells, or killing of host cells by CTLs

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20
Q

What is the trigger for Type 1 hypersensitivity mostly?

A

Environmental Ags, which activate mast cells in an IgE dependent manner

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21
Q

What are the most important mediators of type 1?

A

vasoactive amines, proteases, prostaglandins, leukotrienes, cytokines

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22
Q

What mediator in type 1 causes vascular dilation / permeability?

A

histamine (vasoactive amine)

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23
Q

What’s an important concept associated with type 1?

A

First exposure you most likely aren’t going to have the reaction. it’s the set up.

second is when it explodes with mast cell degranulation

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24
Q

Which cells produce the IgE needed for type 1?

A

Th2

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25
What are 2 major reactions we talked about that are associated with type 1? What is the test that we do to confirm type 1 hypersensitivity? How long does it take?
Asthma Anaphylaxis Allergen Testing --> dermis injection 20-30 minutes
26
What is allergen-specific immunotherapy good for? What is the key cell for this successful healthy immune response?
only way to approach allergic diseases. giving them increasing doses of allergen over time. Foxp3+CD4+CD25+ Treg cells
27
In type 2, which complement is activated? What creates the pro inflammatory response in type 2?
IgG and IgM activate the classical complement pathway IgG binds to Fc receptors on neutrophils and macrophages the ROS and lysosomal enzymes are released
28
What is Autoimmune Hemolytic anemia associated with?
type 2 hypersensitivity?
29
Goodpasture's syndrome?
Type 2
30
What is Grave's Disease?
hyperthyroidism - Abs stimulate the activity of TSH receptors even if there's no hormone around. Type 2
31
Myasthenia Gravis?
inhibit the binding of acetylcholine neurotransmitter to Ach receptor causing the disease
32
What is Type 3 hypersensitivity?
Ab-Ag complexes are formed in the circulation and deposited in blood vessels and other sites. induce vascular inflammation, ischemic damage
33
What's the classical mechanism in triggering tissue damage for type 3?
classical activation of complement
34
What are the 4 diseases that he talked about that are Type 3 hypersensitivities?
Systemic Lupus Erythematosus Post-streptococcal glomerulonephritis Serum Sickness Arthus Reaction
35
How does the Arthus reaction work?
induced by subcutaneous administration of a protein Antigen to a previously immunized animal. it results in the formation of immune complexes at the site of Ag injection, and a local vasculitis
36
What's the difference between Arthus reaction and systemic reactions?
Arthus reactions are localized reactions.
37
What are the main type 4 hypersensitivity reactions that we talked about? What is the mnemonic
KCU is the MRDCPT of KC ``` MS RA Type 1 diabetes Crohn's disease Poison Ivy (contact sensitivity/ dermitis) Tb (chronic infections) ```
38
What is associated with type 4 hypersensitivity reactions?
Macrophages sense the cytokine response that was created by CD4 T cells or CD8 T cells. Th1 or Th17
39
What is Delayed-type hypersensitivity? when does it happen? what are examples of DTH reactions that are microbial based? Contact sensitization? Immunization based?
CD4 T cell activation 24-48 hours after Ag challenge Tb Poison Ivy Diphtheria Toxin/tetanus toxin
40
What is a PPD used for?
Mycobacterium tuberculosis .. this is the tuberculin reaction type 4
41
What are forms of allergic contact dermatitis? what form is it?
Type 4 metals are most common. poison ivy
42
What is granulomatous Inflammation
granulomas have activated macrophages and multinucleate giant cells and lymphocytes (T cells). centra area of necrosis is seen
43
What creates granulomas?
prolonged reactions of th1, macrophage activation and leukocyte recruitment this is due to ion gamma and tnf stimulating macrophages takes days to weeks to make a mature granuloma
44
What is systemic lupus erythematous? diagnostic test?
type 3 hypersensitivity you get rashes, arthritis, glomerulonephritis presence of anti-nuclear abs
45
What is the only disease that has a mix type hypersensitivity reaction? what cells are involved?
RA mediated by both type 2 and 3 antibodies Th1, Th17, B cells, plasma cells, Macrophages
46
What factor is most important in IBS?
TNF
47
what microbes flourish during immunodeficiencies?
opportunistic bacteria
48
What is a PID?
primary immune deficiencies (PIDs)
49
Why are PIDs clinically manifested after 5-6 months?
because maternal IgG is until 6 months. immune deficiencies aren't detected in the newborn yet
50
What is SCID? clinical presentation? what happens to mothers with this?
deficiency in both T AND B AND SOMETIMES NK severe lymphopenia result in abortion because rejection of maternal T cells that cross the fetal circulation in utero
51
What constitutes a SCID without NK cells? why is this? What group of people can be affected?
Adenosine deaminase deficiency (ADA) leads to buildup of toxic products that kill everything boys and girls
52
What constitutes a SCID with NK cells? what is it's role? what is still okay?
Artemis / Rag1 Rag2 deficiency.. Artemis is responsible for recombination and double strand break repair ADA is still fine
53
what is common gamma chain deficiency? what kind of disease is this? What do you see with regards to T, B, and NK
deficiency in T cell development but still functional B cell deficiency hence this is a SCID T-, B+, NK-
54
Where do we see ADA deficiencies? what about Rag1/Rag2/Artemis deficiencies?
In T and B cells at the progenitor level CD4-CD8- cells Pro B cells
55
What is the genetic inheritance of common gamma chain deficiency? What do they present with in their blood?
X linked only B cells, but nonfunctional
56
What is a deficiency of Jak3? genetic inheritance? What kind of disease is this? What signaling is affected? What is present in the blood?
mutation in gene, development of T cell autosomal recessive SCID IL-2 receptor signaling T-, B+, NK-
57
What is DiGeorge Syndrome? what do they present with?
nonfunctional thymus due to deletion of 22q11 chromosome facial abnormalities defect of heart outflow LACK OF T CELLS
58
What is causing an MHC Class I deficiency? What cells should be normal? What kind of hypersensitivity is this?
TAP 1 molecules are not working to transfer stuff to the ER CD4, CD8 doesn't work Type 4
59
Bare Lymphocyte Syndrome involves what? What cells should be normal?
CD8 works, CD4 doesn't MHC 2 isn't working on APCs, but it works on chromosome 6
60
Wiskott-Aldrich syndrome? What is the genetics of this disease? What does it present with? What is the makeup of IgM, IgG, IgA, IgE
Decrease in T cells defect in WASP protein on hematopoietic lineage thrombocytopenia, eczema Low IgM; normal everything else
61
What's a key sign to a baby with Wiskott-aldrich
bleeding from circumcision site, excessive bruising, bloody stool
62
What immunoglobulin is associated with encapsulated bacteria?
IgM
63
IPEX is associated with a mutation where?
FoxP3
64
ALPS (autoimmune lymphoproliferative syndrome) is associated with what? What's the result?
Fas, Fasl, Caspase 8 or (the weird one) Caspase 10 no apoptosis by T effector cells
65
When someone says Bruton Agammaglobulinemia, what gene that's effected and what's the genetics?
BTK X linked
66
If someone said Agammaglobulinemia and female, what are the genes it could be?
BLNK, Ig-alpha chain mu chain delta 5 chain
67
For any form of Agammaglobulinemia, what are the levels of IgG, IgA, IgM?
low to absent
68
What is Common Variable Immune Deficiency? what's the genetics? What are the levels of IgG, IgA and IgM?
hypogammaglobulinemia Low IgG, Low IgA, normal/low IgM
69
What are the two causes to Common Variable Immune Deficiency
B cell formation defect - inherited! Abs against B cells - acquired
70
What happens during an IgA deficiency?
pretty much asymptomatic. other things take over. you just have low IgA
71
What is Hyper IgM syndromes? What levels of B cells do we have?
Impaired Ig class switching and somatic hypermutation Normal peripheral B cells but low memory B cells
72
What are the two ways in which you could have Hyper IgM syndromes?
X-Linked CD40L deficiency Autosomal CD40 deficiency
73
where is CD40 and CD40L located?
CD40 is on the B cell (or other APCs) CD40L is on the T cell
74
What are the levels of IgM, IgG, and IgA of Hyper IgM syndromes?
High IgM, low IgG and IgA
75
What would you see in a person with isolated IgG subclass deficiency?
everything is normal, just IgG2 is lower in kids, or IgG4 may be absent. this really doesn't do much
76
What's the difference between Transient hypogammaglobulinemia and Hyper IgM syndrome?
IgM is normal and IgG / IgA are low.. whereas hyper IgM has higher IgM than normal.
77
Transient Hypogammaglobulinemia is seen in infants why?
Maternal IgG disappears after birth of the baby and intrinsic IgG starts immediately after birth. so you would see low of IgG and IgA. this just needs time to settle itself out
78
Severe Chronic Neutropenia? clinical presentation?
Phagocytic deficiency 500 cells or less per cubic millimeter lower inflammation and increased susceptibility
79
Chronic Granulomatous Disease? what's the deficiency involve what is CGD associated with when it has recurrent infections?
lots of granulomas deficiency of NADPH so no superoxide so no elimination of extracellular pathogens Catalase positive bacteria
80
Myeloperoxidase deficiency is found where?
diabetes patients
81
What is the genetics of G6PD deficiency? what does it cause? which one is it similar to?
X linked anemia forms granulomas like CGD
82
What is Chediak higachi Syndrome?
abnormal giant granules defect in chemotaxis and degranulation patients are toast by 30
83
What are the phases of chediak higachi syndrome?
1 - susceptibility to infection | 2 - accelerated lymphoproliferative syndrome.
84
What do patients with Chediak higachi present with?
No NK activity giant azurophilic cytoplasmic inclusions partial albinism
85
What happens if you have a defect in IFN gamma and IL-12? What is this most susceptible to?
IL-12 is released from Macrophages and DCs and binds to IL-12R on T cells and NK, which release IFN gamma nontuberculosis mycobacteria
86
LAD is the lack of expression of what? what is affected? presentation?
LFA-1 migration of neutrophils to site of infection slow wound healing, failure of pus formation
87
Which disease affects C1, C4, and C2 of classical complement?
systemic lupus erythematosus
88
Which affects C5-C9
Neisseria
89
Factor H deficiency causes what?
hemolytic uremic syndrome and glomerulonephritis
90
What is the feature of C1 inhibitors?
Hereditary angioedema
91
RA is associated with which complement proteins?
C1 or C4
92
What is hereditary angioedema caused by? what does this naturally do? What structures would swell?
deficiency of C1-Inh C1-inh naturally decreases bradykinin levels. if this were not working, bradykinin goes up allowing fluids to pass through vessel walls and create a bunch of swelling swelling of abdominal cavity skin of hands, feet, face airway swelling

HDM Final (3 decks)

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