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;
35 Cards in this Set
- Front
- Back
|
When was an immmunodeficiency first described?
|
as early as 500 b.c.
|
|
|
T/F immunology as science is a new discipline.
|
true, b/c of HIV that we started to learn about immune system.
|
|
|
What are the 2 types of immunodeficiences?
|
Primary/congenital
Secondary/acquired |
|
|
What is described as primary/congenital immunodeficiency?
|
are genetic defects that result in an increased susceptibility to infection
- is frequently manifested in infancy or childhood (because at beginning have mother's ab) - such diseases affect about 1 in 500 people in the US |
|
|
What is describe as secondary/acquried immuno.
|
develop as a consequence of - malnutrition, disseminated cancer, treatment with immunosuppressive drugs, or infection of cells of the immune system.
|
|
|
INtegrity of the immune system is essential for defnese againt infectious organisms and their toxic products
that being said, is the immuen sytem impt. for our survival? |
yes!
|
|
|
T/F Toll-like receptors are highly conserved across widely diverse species
|
true, any loss of function mutation affecting a TLR has negative consequences for survival
|
|
|
What are TLR?
|
on immune cells like centinals (macrophages and leukocytes)
- part of innate immune system |
|
|
Can you survive without an innate immune system?
|
no
|
|
|
Can you survive with damage in adaptive immune system?
|
yes
- affect primary adaptive immune system and thus affect adaptive |
|
|
Where can the system be 'damaged' in primary immunodeficiencies
|
affect primarily one or more components of the immune system, including T/B cells and NK cells as well as phagocytic cells and complement proteins
- or may result from defects in Leukocyte matureation or activation for from defect in effector mechanisms or innate and adaptive immunity. - so pretty much everything can be affects = problem in presence of ag |
|
|
What is the principal consequence of an immunodeficiency ?
|
an increased susceptibilty to infection:
the nature of the infection in a particular pt. depends largely on the component of the immune system that is defective. in other words: the types f recurring infections can predict the type of immunodeficiency - pyogenic - humoral viral - cell-mediated |
|
|
X-linked agammaglobulinemia - XLA - does this effect men or women more commonly?
|
MEN, because it is x-linked.
all ab isotypes are very low - not even IgM or IgD - circulating B cells are usually absent - Pre-B cells are present in reduced numbers in the bone marrow - no germinal centers in LN so they are small (because no B cells) - Thymus architecture is normal, as are the T cell-dependent areas of spleen and LNs |
|
|
What is the defect in Bruton's Agammaglobulinemia?
|
with a loss of function of Bruton Tyrosin Kinase that is impt. for pre-B cell expansion and maturation into Ig-expressing B cells.
|
|
|
What type of Immunodeficiency and what system (humoral or cell-mediated) is Bruton's Agammaglobuloinemia?
|
Humoral - primary deficiency
|
|
|
What is X-linked immunod. with hyper IgM?
|
can only make IgM in high quantities
-very low serum IgG, IgA, and IgE like boys with XLA, pts. with hyper -IgM may become symptomatic during the first or second year of life with recurrent pyogenic infections (humoral) - otitis media, sinusitis, pneumonia, and tonsillitis |
|
|
*What is the diff. between hyper-IgM and XLA?
|
*pts. with XLA, don't have lymphoid hyperplasia and hyper-IgM do have hyperplasia
have very large and palpaple LN |
|
|
What is the defect of hyper IgM?
|
Occurs during isotype switching - typically with B cell, in the beginning have igM on their surface, once they recognize ag, they go through isotype switching and secret ab for the same ag. - cells susceptible to this can't DO this! Can't express CD40 ligand (critical coactviation molecule for B cells) thus, loss of this molecule prevents the T cell from co-stim ag-specific B cells (through CD40)
|
|
|
SO, what does the problem with T cell's not expressing CD40 ligand have to do with B cell isotype switching?
|
B cells are not signaled by the T cell to go through isotype switching and only produce IgM
|
|
|
Deficient _________immunity usually results in increased susceptibility to infection by pyogenic bacteria
|
humoral
|
|
|
What is the treatment for deficiency in humoral immunity?
|
pretty routine - prophylactic ab and/or gamma globulin therapy
|
|
|
Deficient ----------- immunity usually results in increased susceptiblilty to viruses and other intracellular pathogens.
|
cell-mediated
|
|
|
T/F Cell mediated immunity is a little bit "rougher' or more dangerous for the indiv.
|
true, b/c harder to treat, and infections more serious
|
|
|
T/F It is rare that pts. with absolute defects in T-cell function survive beyond infancy or childhood.
|
true
|
|
|
DiGeorge's Syndrome - is what?
|
developmentally-related disease
thymus does not develop ind. with this problem can't mature T cells b/c no where to go after leave bone marrow. Relative increase in B cell function |
|
|
B cell increased numbers in ind. with DiGeorge's syndrome still have impaired B cell function, why?
|
Because B cells need T Cells to work.
|
|
|
A more common X-linked Recessive Severe Combined immunodeficiency Disease - in pop culture, what is this referred to as? why?
|
the bubble boy
- X-linked SCID (XSCID) growth appears normal initially, but extreme wasting usually develops after infections and diarrhea begin - persistent infections with opportunisitc organisms - these infants also lack the ability to reject foreign tissu and are therefore at risk for GVHD (graft vs. host disease) - XSCID pts. have few or no T cellls and NK cells - Pts with XSCID usually have elevated percentages of B cells - but these B cells dont' produce immunoglobulin normally normally, even after T-cell reconstitution by bone marrow transplantation |
|
|
How do you treat Immunodeficiences?
|
Bone marrow transplant - main treatment of choice (XSCID)
passive immunization Current treatments of immunodeficience have two aims: 1 - to minimize and control infections 2 - replace the defective or absent components of the immune system by adoptive transfer and/or transplantation |
|
|
What is an example of secondary/acquired immunodeficiency disease?
|
most commonly recognized: HIV
|
|
|
What is HIV, how does it work?
|
attaches itself to CD4+ receptors on the T cell primarily and to chemokine receptors
|
|
|
What are the most common reservoirs of HIV?
|
CD4+ T cells - primarily infected and release most in blood stream
- macrophages/monocytes - resting/memory CD4+ cells All = plasma Virus |
|
|
What is the progression of the HIV disease?
|
DC if and when they pick up HIV - they can then carry it to the LN and this is where the majority of your B and T cells live and they are then recruited and infection is established. = spread via viremia
- have some control via cytotoxic lymphocytes - so they will be killing infected T cells |
|
|
So, during the progression what two things are seen?
|
Thus 2 things occur:
1. clinical latency - viral cessation (can be trapped in DC and wait for ) 2. full AID response - destruction of the lymphoid tissue. |
|
|
T/F Patients can live longer no than they used to with treatment?
|
yes, see the symptoms much later in life leading to death (years later)
- don't know why it become active again...have inc. loss of T cells and dec. effect of fighting infection |
|
|
SO, what DOES kill the HIV pt?
|
opportunistic infection and diseases because of lack of immune system.
|
