Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Sem 2: Immuno > Immunodeficiency - TOKA > Flashcards

Immunodeficiency - TOKA Flashcards

1
Q

What is immunodeficiency?

A

Failure in humoral or cell mediated limbs of immune system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is primary immunodeficiency?

A

A result of genetic defects in 1 or more components of immune system

  • intrinsic defects in T or B cells

** usually genetic @ birth

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is secondary immunodeficiency?

A

Loss of function of antibody or lymphocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What genetic defects can lead to primary immunodeficiency? (8)

A
  1. Failure of pluripotent cells to differentiate (fetus cant develop)
  2. Failure of lymphoid lineage to differentiate from stem cells
  3. Lack of T cell development
  4. Lack of B cell development
  5. Blockage of B cells to make plasma cells
  6. Failure of plasma cells to make Ig classes
  7. Failure to make neutrophils + macrophages
  8. Failure to make complements components
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What signs are suspected for primary immunodeficiency?

A
  1. Chronic or recurrent infection
  2. Multiple site infection in young
  3. Infection with foreign microorganisms
  4. Failure to respond to Antibiotic or chemo
  5. Lack of response to vax Ag
  6. Leukocytosis / Hypergammaglobinemia
  7. Leukopenia / Hypogammaglobulinemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

How can you diagnose primary immunodeficiency?

A
  1. Necropsy of dead litter mates
  2. Bone marrow / LN biopsy
  3. Detect complement proteins in serum
  4. Screen for infectious dz
  5. Determine CD4/CD8+ ratio
  6. Test for neutrophils/macrophages
  7. Assess type IV hypersensitivity
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What syndrome causes a defect in the LYST gene? What does this lead to?

A

Chediak- Higashi syndrome (CHS)

Leads to formation of large secretory lysosomes in neutrophils, eosinophils, etc. which fuse, rupture, and cause tissue damage

Can cause cataracts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Chediak- Higashi syndrome is a ______ mutation

A

Missense - replaces Histidine with Arginine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

A functional lyst gene is responsible for ___________

A

regulating lysosomal membrane trafficking

moving neutrophils, etc from blood to tissues

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the clinical presentation of Chediak- Higashi syndrome?

A
  • Loss of skin color
  • dilution of hair pigment
  • Cataracts
  • Photophobia
  • Increased susceptibility to infections + tumors bc reduced neutrophils

** - Abnormally bleeding + hematomas @ injection sites

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

A dog comes in for an annual vaccination. After giving the injection, the dog starts bleeding alot at the site of injection. You finally get the bleeding to stop and the dog goes home. The next day the dog comes in with a huge hematoma. What is suspected?

A

Chediak- Higashi syndrome

Abnormal bleeding and hematomas can cause death by acute hemorrhage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

How can you diagnose Chediak- Higashi syndrome?

A

Blood smear of enlarged granules in neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Which cells are dysfunctional in CHS?

A

NK cells, Neutrophils, CD8+ Cytotoxic T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Which autoimmune disease can be seen due to a lack of Integrin CD11b/CD18 (Mac 1) ?

A

Canine leukocyte adhesion deficiency (CLAD) / BLAD in cows

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What happens when a dog has CLAD?

A
  • Neutrophils/Leukocytes dont have integrin so they cant extravasate to the tissues from blood
  • Animal can die due to severe infections
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What levels of neutrophils and eosinophils would you expect to see in the blood of a dog with CLAD and why?

A

Increased neutrophilia

Because neutrophils cant leave blood to go to the tissues bc NO INTEGRIN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What dog breeds are most susceptible to CLAD?

A

Irish Red and White setters

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

CLAD is a _________ mutation

A

Missense - Cysteine is replaced with serine

*** as a result CD11b is not expressed because the Beta chain of CD18 is not expressed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

With canine cyclic neutropenia, there is a defect in the ______ gene which is responsible for ____________

A

AP3B1 gene

Responsible for trafficking of granular proteins like elastase to cytoplasmic granules during HEMATOPOIESIS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the result of Canine cyclic neutropenia?

A

Cyclic arrest of maturing myeloid progenitor cells in bone marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What do you expect to see with neutrophils in Canine Cyclic Neutropenia?

A

Fluctuating #s of neutrophils in peripheral blood

Loss of neutrophils every 11-12 days

  • leads to abnormal imflamm. rxn and recurrent BacT and Fungal infections
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Canine Cyclic Neutropenia results in neutrophils having a reduced __________ activity

A

Myeloperoxidase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is an autosomal recessive disease in Border collies?

A

Canine Cyclic Neutropenia

appears after weaning and causes a gray/silver hair coat

24
Q

Prolonged antibiotic therapy of Canine Cyclic Neutropenia leads to ________

A

Amyloidosis

25
Q

Severe Combined Immunodeficiency Disease (SCID) in horses + dogs is from inheritance of an ______________ trait

A

Autosomal recessive trait

26
Q

SCID is from a defect in ____________

A

DNA- dependent protein kinase catalytic subunit (DNA-PKcs)

27
Q

What disease is caused by a mutation that removes 5 nucleotides leading to a loss of 967 AA?

A

SCID

28
Q

SCID results in

A

***** no recombination of variable regions of TCR and BCR !!!!!

so lymphocytes cant recognize Ag

** lack of sufficient T and B cell production, low numbers in circulation (lymphopenia)

29
Q

Would you expect to see high or low numbers of T and B lymphocytes in the blood of a horse with SCID?

A

LOW NUMBERS of T and B cells in circulation

30
Q

What cells will be functional in a horse with SCID?

A

Neutrophils, NK cells, and monocytes

Bc these dont need recombination receptors!

31
Q

How can you diagnose SCID in horses?

A
  1. Low circulating lymphocytes
  2. Lack of IgM in serum before suckling
  3. Hypoplasia of primary and secondary lymphoid organs

** no germinal centers or PALS in speen!

32
Q

Which dog breed is susceptible to SCID?

A

Jack russels

33
Q

SCID in dogs can cause?

A
  1. Lymphopenia
  2. Agammaglobulinemia (No Ab)
  3. Hyperplasia of primary and secondary lymph organs
34
Q

What immunodeficiency is seen exclusively in males and is most common in corgis and bassets?

A

X-SCID

X-linked severe combined immunodeficiency

*** Lack of lymphnodes!!!!

35
Q

X-SCID is from a mutation in the gene encoding _________

A

y-chain of the IL-2 receptor

**lymphocytes are not reactive in the presence of IL-2 :(

36
Q

X-SCID is a _______ type of mutation

A

Nonsense muation

  • results in STOP codon that produces a non functional protein
37
Q

What cell is unaffected by X-SCID?

A

B cells will be present - but no class switching to IgG or IgA

38
Q

How will X-SCID affect antibodies?

A

Normal IgM but reduced or lack of IgG and IgA

cant Ig class switch

39
Q

What is the most common selective Ig isotype deficiency?

A

IgA deficiency

40
Q

What Ig deficiency can be found in King charles spaniels?

A

IgG deficiency

41
Q

Selective IgA deficiency can lead to _______

A

Antiobiotic resistance

** NOT BECAUSE OF LACK OF IgA
** DUE TO constant bacT infections with continuous treatment

42
Q

Canine distemper virus attacks __________

A

T and B cells in tonsils, bronchial LNs and blood

** has the same CD150 receptor as lymphocytes - binds and destroys them

43
Q

FeLV has a tropism to ________

A

lymphoid tissue

44
Q

FIV acts by

A

Activating T regs to suppress immune system + increase IL-10 production

Reduces IL-2 and CD4+ T cells

45
Q

FeLV acts by

A

-Attacking T cells + reducing # of CD4+ T cells

  • Wasting syndrome - atrophy of thymus
  • Atrophy or hyperplasia of lymphoid tissue
  • Supresses immune system
46
Q

FIV binds to _______ on activated CD4+ T cells

A

CD134 and CD184

47
Q

Canine distemper virus binds to ____ on activated T cells and leads to virus replication and destruction of the cell

A

CD150

48
Q

Viruses can damage primary or secondary lymphoid organs leading to _________

A
  1. Lymphopenia
  2. Reduction of lymphocyte reactivity
  3. Hypogammaglobinemia
49
Q

Secondary immunodeficiences can be due to:

A
  1. Infectious agents like viruses (FIV, FELV)
  2. Toxins
  3. Stress
  4. Malnutrition
  5. Old age (immunosenescence)
50
Q

Common Variable Immunodeficiency (CVID) can lead to

A

B cells not producing sufficient Ig

This can cause recurrent infections

51
Q

________ defect appears in order horses, over 4 years of age and is due to lack of stimulation of B cells by CD4+ T cells

A

Common variable immunodeficiency (CVID)

52
Q

Why does antibiotic resistance occur with selective IgA deficiency in dogs?

A

Because of constant bacT infections and continuous treatments with antibiotics

53
Q

In FIV, what cell type is reduced?

A

CD4+ T cells

54
Q

Immunodeficiences associated with innate immunity may involve defects in __________

A
  • Lack of Phagocytosis
  • Lack of complement
  • Dysfunctional NK cells
55
Q

With SCID, why are foals born healthy but develop health problems around 2 months of age?

A

Because they have passive immunity from mom, but that wears off and the baby has T and B cells with no recombination (No Ag specificity) and cant recognize Ag

Sem 2: Immuno (17 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions