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;
76 Cards in this Set
- Front
- Back
|
what are 2 T independent antigens?
|
polysaccharides
lipopolysaccharides |
|
|
what cytokines do Th1 cells produce? (3)
|
IFN-gamma
IL-2 TNF-beta |
|
|
what cells to Th1 cells help? (3)
|
macrophages
NK cells CTLs |
|
|
what cytokines to Th2 cells produce? (6)
|
IL-4, 5, 6, 10, 13
THF-beta |
|
|
what is IL-4 involved in?
|
class switching to IgE
|
|
|
what is IL-5 involved in?
|
class switching to IgA
|
|
|
why is IL-6 known as the B cell differentiating factor?
|
it is critical in differentiating B cells into plasma cells producing antibodies
|
|
|
what do virally infected cells produce? (2) and what does this do?
|
IFN-alpha and beta
upregulate the endonuclear system to destroy viral RNA and EF-2 in their own cell and neighboring cells |
|
|
if the innate response to viruses fail, what comes into play?
|
CD8 cells
|
|
|
why is the virus not getting killed in AIDS if there are more CD8 cells?
|
they need T helper cells to work
|
|
|
if a patient is repeatedly coming down with viral infections, what do they likely have?
|
CD4 deficiency
|
|
|
what type of bacteria will produce pus?
|
capsular - klebsiella, staph, etc
|
|
|
what are 3 examples of pyogenic infections?
|
pneumonia
otitis media sinusitis |
|
|
what mutation is responsible for XLA and how?
|
mutation in the btk (bruton's tyrosine kinase) gene
required for the maturation of B cells so without Btk, growth is arrested at the pre-B stage |
|
|
if a cell is said to have cytoplasmic mu, what is it?
|
a pre-B cell
|
|
|
how is XLA inherited?
|
x-linked
|
|
|
what is the typical patient in XLA?
|
baby boys about a year old
|
|
|
why do XLA patients not present until after 1 year of age or so?
|
because prior to this they have been protected by maternal immunoglobulins
|
|
|
what will peripheral blood cultures for XLA patients show? (3)
|
very few or no B cells in the blood/lymphoid tissue
No IgM, IgA, or IgE (paradoxically there is some IgG) plasma cells are absent |
|
|
what will be normal in XLA patients?
|
T cell mediated responses
|
|
|
why will XLA patients usually have recurrent respiratory tract infections?
|
these are locations where bacteria are colonized anyway
|
|
|
what are 6 common pathogens in XLA?
|
haemophilis influenza
strep pneumonia strep pyogenes staph aureus enteroviruses giardia lamblia |
|
|
what is the #1 water borne pathogen in the US?
|
giardia
|
|
|
why will females rarely suffer from XLA
|
X-inactivation is not random in females because the defective cells that are not inacitvated anyway will not move past the pre-B stage and thus not be seen
|
|
|
what do B cells express? (3)
|
CD19, 20, and 21
|
|
|
what is CD21
|
receptor for EBV
|
|
|
what CD will XLA patients be lacking?
|
CD19
|
|
|
what expresses CD16?
|
NK cells
|
|
|
what is expressed by NK cells?
|
CD16
|
|
|
what expresses CD14?
|
macrophages
|
|
|
what is expressed by macrophages?
|
CD14
|
|
|
what is CD14 also called?
|
LPS receptor
|
|
|
why is the classic complement pathway the last to be activated?
|
it is activated by antibodies, which take about a week to develop
|
|
|
how do you treat XLA?
|
periodic large doses of gammaglobulin
treat with antibiotics as needed |
|
|
what is the most common type of immunodeficiency?
|
IgA subclass deficiency
|
|
|
what patients will usually be asymptomatic?
|
IgA or IgG subclass deficiency
|
|
|
how do you treat IgA/IgG subclass deficiency?
|
periodic large doses of gammaglobulin
treat with antibiotics as needed |
|
|
how will IgA subclass deficiency usually present?
|
as anaphylactic shock when the patient is exposed to normal blood with IgA in it
|
|
|
what is there a high incidence of with IgA deficiency? (2)
|
allergic disease and autoimmune diseases (especially RA and SLE)
|
|
|
what immunoglobulin is formed via alternative splicing?
|
IgD
|
|
|
what causes the problem with hyper IgM syndrome?
|
T cells not expressing the CD40L (CD40-CD40L interaction is critical for class switching)
|
|
|
what is critical for class switching?
|
CD40-CD40L interaction
|
|
|
what immunoglobulins will not be found with hyper IgM syndrome?
|
IgA, IgG, IgE
|
|
|
what are absent in hyper IgM syndrome? (2)
|
germinal centers in the lymph nodes and other secondary lymphoid organs
|
|
|
what are 2 things IgM does very well?
|
scavenge for antigens
activate complement |
|
|
what can IgM not do?
|
opsonize because it is in a pentamer form
|
|
|
what causes neutropenia in hyper IgM syndrome?
|
lack of macrophage activation depletes GM-CSF which is a growth factor for neutrophils
|
|
|
what other cell types are expressed CD40 that will suffer in hyper IgM syndrome?
|
macrophages
|
|
|
what 3 things are required in class switching?
|
B cell, T cell, cytokines produced by Th2 cells
|
|
|
what do you need to switch from IgM to IgE?
|
IL4
|
|
|
what do you need to swtich from IgM to IgA?
|
IL-5
|
|
|
what does the immunoglobulin become in the absence of IL-4 and IL-5?
|
IgG
|
|
|
what does IgG become in the absence of IFN-gamma
|
IgG2 or IgG3
|
|
|
how can you differentiate hyper IgM syndrome from XLA?
|
hyper IgM syndrome will suffer from both pyogenic and opportunistic infections
XLA will suffer from only pyogenic |
|
|
what will cultured T cells show with hyper IgM syndrome?
|
CD40 will not bind to CD40L
|
|
|
how is hyper IgM syndrome inheritied?
|
X-linked
|
|
|
what is another way to get hyper IgM syndrome in which T cells function fine?
|
defective AID or UNG enzymes
|
|
|
what is AID also responsible for in B cells and what will this lead to?
|
somatic hypermutation so without AID cells cannot undergo affinity maturation and the only response is proliferation leading to lymphadenopathy
|
|
|
what causes lymphadenopathy in hyper IgM syndrome due to AID?
|
B cell proliferation, which is the only response available because somatic hypermutation is unavailable
|
|
|
how is hyper IgM syndrome due to AID inheritied?
|
autosomal recessive
|
|
|
what type of infections will hyper IgM syndrome with AID patients suffer from?
|
pyogenic, not opportunistic because defect is only in B cells
|
|
|
what is the problem in common variable immunodeficiency?
|
T cells not providing help
|
|
|
what is CVID often due to?
|
pernicious anemia
|
|
|
who will present with CVID?
|
both males and females later in life
|
|
|
what is a common infection in CVID?
|
giardia
|
|
|
how is CVID different from XLA?
|
will find B cells in the peripheral blood, unlike in XLA
|
|
|
what is transient hypoglobulinemia of infancy?
|
the period in which neonates do not produce their own antibodies is extended for up to 3 years but resolves completely
|
|
|
how is SCID inherited?
|
X linked or autosomal recessive
|
|
|
what are 3 cardinal features of SCIDs?
|
lymphocyte deficiency with a failure of the thymus to develop
profound deficiency in cell-mediated immunity recurrent infections in early life (vs XLA) |
|
|
what will SCIDs patients ususally suffer from? (4)
|
diarrhea
pneumonia oral thrush FTT |
|
|
what will cause diarrhea in SCIDs patients? (2)
|
rotavirus or GI bacteira
|
|
|
what will cause pneumonia in SCIDs patients? (3)
|
pneumocystic carini
pseudomonas CMV |
|
|
what will cause oral thrush in SCIDs patients? (2)
|
candidia albicans
varicella |
|
|
what will be the lymphocyte level in SCIDs?
|
<3000
|
|
|
how will the thymus appear in SCIDs?
|
fetal appearance
will not contain a cortex or medulla differentiation |
|
|
what is X-linked SCIDS due to and what ramifications doe this have?
|
defect in the gamma chain of IL-2 receptor
this chain is also shared by IL-4, 7, 9, 11, and 15 so these receptors will also be damaged |
